tetralogy of fallot case study

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Surgical repair of tetralogy of Fallot in a 78-year-old woman: a case report

• Yuzo Katayama   ORCID: orcid.org/0000-0002-2206-8212 1 ,
• Sho Isobe 1 ,
• Tsukasa Ozawa 1 &
• Takeshiro Fujii 1  

Journal of Medical Case Reports volume  18 , Article number:  128 ( 2024 ) Cite this article

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Tetralogy of Fallot is a congenital heart disease mostly diagnosed and treated in early childhood. However, there are some adult cases receiving treatment.

Case presentation

We describe a 78-year-old Japanese woman who presented with severely hypertrophic right ventricle, ventricular septum defect, overriding aorta, and severe infundibular stenosis in the right ventricular outflow tract. As hypoxemia was mild and daily exertion was sufficiently possible, home oxygen therapy was introduced. After 1 month, she was referred because of a positive blood culture. The blood culture test was positive four times, therefore, the antibacterial drug was administered according to active infective endocarditis. SpO 2 repeatedly decreased during hospitalization, thus oxygen was needed. As there were infective endocarditis onset and progressive hypoxemia, we planned a surgical correction.

Tetralogy of Fallot was diagnosed and successfully treated with complete surgical correction, and the development of infective endocarditis was the definitive indication for surgery at this late age.

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Early surgical correction has been established as the best treatment for tetralogy of Fallot (TOF). Without surgical correction, most patients die during childhood, making adult patients with uncorrected TOF rare. Additionally, surgical indications for untreated adult TOF are mostly due to progression of heart failure [ 1 ]. We report an extremely rare case in which intracardiac repair was performed on an elderly cyanotic patient with infective endocarditis.

Presentation of case

The case involved a 78-year-old Japanese woman diagnosed with ventricular septal defect (VSD) during her elementary school days. In her 40s, she felt exertional dyspnea fatigue, but was followed up without any restrictions. At 77 years, she consulted our adult congenital heart disease clinic for increasing symptoms since her 70s. She had a history of hypertension in her 40s and was taking oral calcium channel blockers (nifedipine; 10 mg) and angiotensin receptor blockers (ARBs; candesartan cilexetil; 4 mg) prescribed by a family doctor, and no other previous medical history. Although she had one pregnancy and birth, there were no special events when she gave birth. There was no history of smoking and drinking and no special cardiovascular events in her family history. She has been working as a typist in an office since her 20s.

Electrocardiography showed sinus rhythm, right bundle-branch block, and right ventricular hypertrophy with secondary ST–T wave abnormalities. Chest radiography showed an enlarged cardiac silhouette and dilated pulmonary arteries. Transthoracic echocardiography and contrast computed tomography (CT) revealed overriding aorta, right ventricular outflow tract (RVOT) stenosis (Fig.  1 ), and right ventricular hypertrophy in addition to VSD. The final diagnosis was tetralogy of Fallot (TOF). As hypoxemia was mild and daily exertion was sufficiently possible, home oxygen therapy was introduced after comprehensively explaining the disease and its prognosis.

Transthoracic echocardiography revealing a ventricular septum defect in the perimembranous region and right ventricular outflow tract stenosis. A Color Doppler echo showing the short axis view. B Peak pressure gradient was 82 mmHg

After 1 month, she was referred from the family doctor and hospitalized because of a positive blood culture test. The physical findings on admission were as follows: body temperature 38.1 ℃, blood pressure 135/71 mmHg, pulse rate 71 beats per minute and regular, and SpO 2 87%. Systolic murmur was heard at the left sternal border of the second intercostal space, not rales. No special findings were found on the palpebral conjunctiva, fingers and toes, palms, and soles of the feet. No neurological abnormal findings were observed.

Laboratory findings on admission were as follows: WBC 11,000/μL, Hb 17.5 g/dL, Hct 54%, Plt 19.7/μL, CRP 4.1 mg/dL, AST 24 IU/L, ALT 13 IU/L, LDH 240 IU/L, Na 139 mEq/L, K 3.9 mEq/L, Cl 105 mEq/L, TP 7.0 g/dL, Alb 4.1 mg/dL, BUN 14 mg/dL, Cr 0.59 mg/dL, eGFR 73.1, BNP 88.4 pg/dL, urine specific gravity 1.015, PH 7.0, white blood cell count 0. The blood culture test was positive four times (Lactobacillus rhamnosus) and antibiotic susceptibilities of this bacterium were as follows; penicillin G > 2, aminobenzylpenicillin > 2, ceftriaxone 0.5, gentamicin 1, minocycline 0.25, vancomycin > 16, teicoplanin 16, and levofloxacin 2. Therefore, continuous intravenous administration of ceftriaxone 2 g/day was administered for 6 weeks according to active infective endocarditis. At the time of admission, the patient had a fever of 38 °C, but it remained at 36 °C 3 days after the start of administration. The inflammation data also normalized and negative blood cultures were confirmed twice 3 weeks after starting administration. SpO 2 repeatedly decreased to 80% during hospitalization, thus oxygen was started and 3 L was needed to maintain around 90%. Transthoracic echocardiography, thoracic and abdominal CT, head magnetic resonance imaging (MRI), and upper and lower gastrointestinal endoscopy were also performed, but the site or route of infection could not be identified.

Cardiac MRI revealed severe infundibular stenosis and tiny pulmonary valve regurgitation (Additional file 1 : Videos S1 and S2), normal right ventricular contractility, 90% normal left ventricular volume, and 0.67 Qp/Qs (pulmonary blood flow/systemic blood flow ratio). As there were progressive hypoxemia and infective endocarditis onset, we planned a surgical correction after infection control of infective endocarditis.

The operation was performed on full cardiopulmonary bypass. The hypertrophic muscle obstructing the RVOT was resected up to 20 mm and the VSD was closed with a patch. A perforation was found in the left semilunar cusp of pulmonary valve and the lesion accompanied by changes in curability was closed directly. The perforation may have been the source of infection. Intraoperative transesophageal echocardiography showed improvement in the RVOT to 2.5 m per second.

Although all follow-up blood culture tests after surgery were negative, ceftriaxone was administered for 4 weeks. The preoperative BNP level of 79 pg/dL increased to 727 pg/dL on day 8 postoperation, but improved to the same preoperative level after oral diuretic discontinuation, and echocardiography revealed normal cardiac function. The patient was discharged on day 35 postoperation at New York Heart Association (NYHA) class I after removing oxygen. It has now been 2 years since the operation, and she is still doing well.

Discussion and conclusion

Early surgical correction has been established as the best treatment for TOF. Without surgical correction, most patients die during childhood [ 1 ], making adult patients with uncorrected TOF rare. However, there was a report of uncorrected TOF in the oldest patient aged 86 years [ 2 ].

There have been reports of adults with tetralogy of Fallot developing infective endocarditis and brain abscess, but surgical intervention has been avoided [ 3 , 4 ]. There have only been two reports of repair surgery for tetralogy of Fallot in elderly patients aged 75 years or older, but in both cases the chief complaints were cyanosis and dyspnea [ 5 , 6 ]. This case of a 78-year-old patient was an extremely rare case in which surgical intervention was indicated due to cyanosis and infective endocarditis.

Most patients with TOF have cyanosis from birth or the first year of life because of right-to-left shunting. If resistance to flow through the RVOT is less than resistance to flow through the aorta, there will be left-to-right shunt flow across the VSD without peripheral cyanosis and there will be a late presentation similarly to our case. Additionally, the patient has suffered from hypertension since her 40s and had high peripheral vascular resistance, which was one possible reason for the delayed cyanosis appearance. Furthermore, hemodynamic evaluation was performed only with four-dimensional (4D) flow MRI, and blood flow evaluation via collateral blood circulation was not considered. Therefore, there may be a discrepancy with the evaluation by cardiac catheterization. Considering the preoperative clinical findings and postoperative left heart failure, we believe that there may have been a large amount of blood flow from small collateral circulation channels.

There have been several reports on the surgical outcome of intracardiac repair for adult TOF. Sadiq et al . showed acceptable morbidity and mortality rates with good long-term surgical outcome [ 7 ], whereas Hörer et al . showed a high early mortality rate (15.4%) [ 8 ]. Opinions were also divided on the use of transannular patching (TAP), with Sadiq et al . concluding that TAP is not a risk factor, whereas Hörer et al . concluded that TAP is a risk factor [ 7 , 8 ]. Considering the patient’s life expectancy and the preoperative pulmonary valve annulus system being 20 mm, we decided to form an outflow tract of up to 20 mm without TAP.

Despite the sufficient preoperative left ventricular volume and the restriction of RVOT formation, the increase in the left ventricular volume and pleural effusion after surgery required control of left heart failure. Although premised on improving hemodynamics, it appeared that the strategy for outflow tract formation similar to that in childhood should be reconsidered for the elderly. In our case, we performed complete surgical correction of TOF at 78 years of age, and the development of infective endocarditis was the definitive indication for surgery at rare old age.

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Abbreviations

Ventricular septal defect

Right ventricular outflow tract

• Tetralogy of Fallot

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Acknowledgements

We thank Dr. Edward Barroga ( https://orcid.org/0000-0002-8920-2607 ) for editing the manuscript.

This study was not funded by any agency or institution.

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Division of Cardiovascular Surgery, Department of Surgery, Toho University Omori Medical Center, 6-11 Omori-Nishi Ota-Ku, Tokyo, 143-8541, Japan

Yuzo Katayama, Sho Isobe, Tsukasa Ozawa & Takeshiro Fujii

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YK drafted the original manuscript. SI, TO, and TF substantially contributed to the revisions of the manuscript drafts. All authors have approved the submitted version of the manuscript and agree to be accountable for any part of the work. All the authors read and approved the final manuscript.

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Supplementary Information

Additional file 1: video s1:.

Magnetic resonance imaging scan of the heart revealing right ventricular outflow tract stenosis (wall shear stress: RA + RV + PA). Video S2: Magnetic resonance imaging scan of the heart revealing right ventricular outflow tract stenosis (energy loss: RA + RV + PA).

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Katayama, Y., Isobe, S., Ozawa, T. et al. Surgical repair of tetralogy of Fallot in a 78-year-old woman: a case report. J Med Case Reports 18 , 128 (2024). https://doi.org/10.1186/s13256-024-04414-5

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DOI : https://doi.org/10.1186/s13256-024-04414-5

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Pregnancy outcomes among 31 patients with tetralogy of Fallot, a retrospective study

• Kana Wang 1 , 2 ,
• Junguo Xin 3 ,
• Xiaodong Wang 1 , 2 ,
• Haiyan Yu   ORCID: orcid.org/0000-0002-1656-4906 1 , 2 &
• Xinghui Liu 1 , 2  

BMC Pregnancy and Childbirth volume  19 , Article number:  486 ( 2019 ) Cite this article

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Tetralogy of Fallot (TOF) is a severe type of congenital heart disease (CHD) and it confers substantial risk to mother and fetus for pregnant women. However, the outcome of pregnancy in women with TOF has not been well studied.

Women with TOF who have been seen and/or delivered at our tertiary-care hospital between April 2008 and January 2018 were retrospective reviewed.

A total of 31 pregnant women with TOF were identified during ten-year period. Among these patients, cardiac defects remained uncorrected in 12 women and were surgically repaired in 19 women. The frequency of miscarriages, premature birth, and the percentage of neonates of small for gestational age (SGA) were greater in the uncorrected group than the surgically repaired group (16.67% vs 0, 50% vs 5.26, 41.67% vs 10.53% respectively). The neonatal mortality and fetal mortality were not observed in the surgically repaired group, but were observed in the uncorrected group [3.23% (1/31) and 6.45(2/31) respectively]. Furthermore, the obstetric and cardiac complications in the two groups were stratified and analyzed.

Conclusions

Surgical correction of TOF is associated with improved maternal and perinatal outcome. However, pregnancy in women with uncorrected TOF was still seen and it was observed at a rate of 1.4/10,000 in our medical center during ten year period. The high degree of ventricular dilatation heart, high functional classifications, serious cardiac arrhythmias and pulmonary hypertension appeared to be associated with maternal and neonatal risks.

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Tetralogy of Fallot (TOF) is a severe type of congenital heart disease (CHD) with an incidence of 10% in all reported CHD, which is characterized by four components: large ventricular septal defect (VSD), overriding aorta, right ventricular hypertrophy, and right ventricular outflow tract obstruction [ 1 ]. In the past decades, the overall prognosis of CHD has been greatly improved and many patients could reach adulthood because of early diagnosis and timely therapy. However, TOF as a complex and cyanotic type of CHD, its prognosis remain poor, especially in those who did not undergo surgical repair, and it remains the leading cause of indirect maternal mortality among CHD patient with pregnancy. There are great variations in cardiac complication rates for maternal mortality among published studies for TOF patients with pregnancy, it ranged from 0 to 17.5% [ 2 ]. It has been thought that it is approximately 1/10,000 in developed countries and may be up to 25 times higher in the developing countries [ 3 ].

After surgical repair, the majority of young women with TOF could survive into their reproductive age. Without repair, TOF patients rarely reach childbearing age and get pregnant. Since pregnancy introduces extra load on the heart, and can damage cardiac functions, resulting in the increase in both maternal and perinatal morbidity [ 4 , 5 ]. Previous studies have demonstrated that cardiac and obstetric complications are more likely to occur in patients without surgical repair [ 6 , 7 , 8 ]. The most common cardiac complications include progressive dilatation of the right ventricle and ventricular failure, thromboembolism, atrial and ventricular arrhythmias, progressive aortic root dilatation and endocarditis [ 1 , 9 ]. The common obstetric complications include the increased risk in miscarriage, premature birth, and low birth weights, postpartum hemorrhage, paradoxical embolism, thromboembolism, congestive cardiac failure, infective endocarditis, and arrhythmias [ 6 ]. Among these complications, the pulmonary hemorrhage, brain abscess and thromboembolism have been thought to be the most common causes of death [ 1 , 10 ].

To date, the outcomes of pregnant women with TOF have not not well characterized due to limited number of studied cases and lack of data for close follow-up. Therefore, the management of TOF pregnant women remains challenging. Here, we present 31 cases of pregnant women with or without surgical repair for TOF.

This is a retrospective study with an approval from the Institutional Review Board of West China Second University Hospital. Between April 2008 and January 2018, a total of 85,184 pregnant women have been seen and gave birth in West China Second University Hospital. A total of 31 pregnant women with TOF were identified, and further reviewed. During analyses, we compared the overall clinical characteristics, cardiac and obstetric complications, outcome of pregnancy complications between the corrected and the uncorrected groups. In addition, we analyzed the maternal hemodynamic features and obstetric outcomes in each individual patients with uncorrected TOF.

All pregnant women with or without surgical repair were evaluated by echocardiography, electrocardiography (ECG), and routine clinical examinations such as blood pressure (BP), and heart rate (HR). Hypertension, diabetes mellitus, and other pregnancy associated diseases were taken into consideration in the clinical characterization of the patients.

Surgical correction for TOF

Among 31 TOF pregnant patients, 12 cases underwent cardiac surgery before juvenile age (< 18 years old), and 6 cases before 25 years old. The median age for cardiac surgery was 15.52 years. The longest interval from the surgical correction to pregnancy was 29 years and the shortest was 1 year. The average interval was 11.84 years. Except one patient received two cardiac operations (cardiopulmonary correction with pulmonary artery valve replacement at 11 years before pregnancy, and atrial septal defect reparation with tricuspid valvuloplasty at 3 years before pregnancy), the remaining studied patients had one-time cardiac surgery.

Echocardiography

During the past ten years, echocardiography for the detection of CHD and the assessment of haemodynamic status has become mature. All of our patients have been evaluated with this reliable technique. The anatomic feature of TOF including the overriding aorta, anterior deviation of the outlet septum, pulmonary stenosis and right ventricular hypertrophy have been identified and characterized by echocardiography in all studied patients [ 1 ]. The diagnosis of TOF by echocardiography has been made by the presence of a ventricular septal defect and a large overriding aorta, as well as the haemodynamic deviation for valvar functions (right ventricular pressure, ventricular dimensions, and ventricular function).

Electrocardiogram

Arrhythmia may become manifest during pregnancy with TOF and other CHD. The 24-h ambulatory ECGs were performed in patients when they had abnormal ECG pattern or in a setting in which the patient was prone to the development of arrhythmia, such as the presence of abnormal electrolyte level.

Pregnancy data

Data related to pregnancy in this study were mainly collected around the delivery. These data included gestational-age of newborn at birth, type of delivery, blood loss at delivery, birthweight, Apgar score and postpartum hemorrhage. Fetal and neonatal echocardiographic data have also been collected for evaluation of CHD.

Descriptive statistics, such as frequency, percentage, mean, standard deviation (SD), and the range were used for the presentation of variables. The normally distributed variables were presented as Means ± SD and they were compared using Student’s t-test for the differences between groups. The distribution of blood loss was presented in the format of Median ± IQR (interquartile ranges) and was compared using Mann–Whitney U test. Categorical variables, such as clinical characteristics and complications, were expressed as proportions and compared using Chi-square test or Fisher’s exact probability test. All statistical analyses were performed in SPSS (version 20.0). An alpha of 0.05 is used as the cutoff for significance.

Patient characteristics

Thirty-one pregnant women with TOF were identified and analyzed in this study. The sociodemographic information for these patients, including age, region, education degree, gravidity and parity, were collected and summarized in the Table  1 . The age of the studied patients ranged from 19 to 39 years. Specifically, the age was between 22 and 35 years (median: 28 years old) in the surgically repaired group and between 19 and 39 years (median age was 26.5 years old) in the uncorrected group. The majority of the patients who did not undergo TOF repair surgery came from remote rural areas and did not have higher education. The frequency of abortion and gestation were higher in uncorrected group than in the surgically repaired group. Of note, 94.74% (18/19) pregnant women in the corrected group were primiparity.

The surgically procedures for TOF repair included the closure of the VSD by insertion of transannular patches, rendering the pulmonary valves incompetent, right ventricle infundibulectomy, and transannular enlargement of the right ventricular outflow tract, as detailed in the Method section. Despite of variation of the surgical procedures, the repair operations reached the goal for correction in the majority of patients, except two patients who still had residual shunt after ventricular patch.

Cardiac and obstetric complications in two groups

Cardiac and obstetric characteristics in 31 pregnant women with TOF are summarized in Table  2 and Table  3 , respectively. Obstetric and cardiac complications were observed more frequently in the uncorrected group. The rate of prematurity was significantly higher in the uncorrected group than in surgically repaired group (50% vs 5.26%, respectively, P  = 0.007). The frequency of spontaneous abortion was also greater in the uncorrected group than in the surgically repaired group (16.67% vs none, respectively). The percentage of small-for-gestational-age newborn was 41.67% in the uncorrected group and 10.53% in the surgically repaired group ( P  = 0.078). The mean of neonatal birth weight was significantly lower in the uncorrected group than in the surgically repaired group ( P < 0.001). However, the total days of stay in hospital and blood loss at delivery were similar in both groups ( P  = 0.866 and 0.586 respectively). The rate of postpartum hemorrhage was 10.53% in the surgically repaired group and 8.33% in the uncorrected group [due to placenta previa in both groups (2 vs 1 case, respectively), instead of abnormal blood coagulation. The CHD [Patent ductus arteriosus (PDA) and patent foramen ovale (PFO)] was observed in one baby from the surgically repaired group (Table 2 ).

Most of patients in the surgically repaired group had good cardiac functional status with NYHA class of I-II, which was significantly better than uncorrected group (63.16% vs 16.67%, respectively, P  = 0.031). Ten (83.33%) cases had cardiac function NYHA class III or higher in the uncorrected group. Cardiac diameter and ventricular systolic function were assessed in all patients. The moderate or severe right ventricle dilation was found in all uncorrected patients (12 cases, 100%), but only in 9 cases (47.37%) in the surgically repaired group. The right ventricular dilation was absent or in a very mild degree in the remaining 10 corrected cases (52.63%) ( P  = 0.004). Of note, one uncorrected patient showed a limited systolic function (EF = 31%, FS = 16%) and pulmonary arterial hypertension (PAH). In the current study, the most common complications attributable to TOF were outflow tract and valve condition, ventricular shunting with ventricular septal defect and arrhythmias. Pulmonary valve stenosis was seen in all uncorrected patients (100%) and in 4 cases (21.05%) of corrected TOF ( P  < 0.001). Pulmonic regurgitation was found in 6 (31.58%) in the surgically repaired patients but none in uncorrected patients ( P  = 0.059). According to the ECGs, ventricular hypertrophy was present in all uncorrected patients (100%) but only 5 cases in surgically repaired groups (26.32%, P  < 0.001). Following surgical repair, ECG became normal in 5 patients (26.32%), but complete right bundle branch block (CRBB) have been still detected in 9 (47.37%) corrected patients. No other abnormal ECG was found between two groups (Table 3 ).

Maternal and perinatal outcomes in the women with uncorrected TOF

The detailed clinical information concerning maternal and perinatal outcomes in the women with uncorrected TOF is summarized in Table  4 . Among them, five women (41.67%) were primigravidae (G1P0), the remaining 7 women had a history of abortion or childbirth. Except that one patient with intrauterine fetal death who underwent transvaginal complete curettage of uterine cavity, the mode of delivery in remaining patients was cesarean section (C-section). During the operation, 6 patients were under general anesthesia, 4 patients under epidural anesthesia, and 1 patient under combined spinal-epidural anesthesia. Regarding the obstetric complications related to the prematurity including miscarriages, premature labor, small for gestational age (SGA) and low birth weight infant at term (LBWI), half of women (50%) were due to premature rupture of fetal membranes (PROM) and/or prenatal hemorrhage. The rate of SGA was 41.67% ( n  = 5). One case with NYHA class IV complicated with severe pulmonary hypertension and limited systolic function had to terminate pregnancy at 27 + 6 weeks. That patient was transferred to the Department of Cardiology for further treatments and survived. Unfortunately, her baby died at the 7th day after birth due to neonatal pneumonia, septicemia and intracranial hemorrhage. The maternal mortality in this study was none. Neonatal mortality and fetal mortality were observed only in the uncorrected group with a rate of 3.23% (1/31) and 6.45% (2/31), respectively (Table 4 ).

Tetralogy of Fallot is a severe type of CHD and it is characterized by the hemodynamic alterations due to anatomic abnormality, with varied degree of comprised cardiac functions, including ventricular dysfunction, right ventricular systolic dysfunction, right ventricular dilation, outflow tract obstruction, and pulmonary hypertension [ 11 ]. After surgical repair of TOF, the majority of young women could survive into their reproductive age. But it is generally known that heart disease constitutes a leading nonobstetric cause of maternal mortality, especially in patients with the cyanotic and complex shunt lesions [ 6 ]. Previous studies for pregnant women with TOF showed that pregnancy confers a considerable risk to these patients. The adverse cardiovascular events may be associated with right ventricular dysfunction, severe pulmonary hypertension, and severe pulmonic regurgitation with RV dysfunction [ 3 , 11 ]. It has been demonstrated that abnormal uteroplacental Doppler flow (UDF) was associated with right ventricular function parameters, suggesting that maternal cardiac dysfunction contributes to defective placentation and/or placental perfusion, which subsequently increases the incidence of obstetric and neonatal complications [ 12 ].

With emerging more advanced diagnostic and therapeutic approaches, the overall prognosis of TOF patients has been remarkably improved in the past decades. However, we occasionally encountered that women with uncorrected TOF got pregnant. Due to the extra cardiac load during pregnancy, women with uncorrected TOF had much higher maternal and perinatal morbidity compared to general population [ 1 , 2 , 3 , 4 , 5 , 9 ]. These cases are relatively rare and almost all of published studies for these patients in the literature were case reports. Thus far, no standard regimen has been established to manage these patients. Accordingly, it will be extremely beneficial to study these cases in a comprehensive manner. This study was for the first time to collect over 30 cases of TOF patients from a single center and perform comparative analysis between uncorrected cases and surgically repaired cases.

It is worthwhile to stress that right ventricular dilation were found in all uncorrected patients but in less than half patients in the surgically repaired group. In addition, no incidence of thromboembolism and endocarditis were found in studied subjects, which may explain the overall good clinical outcomes in spite of different degrees of arrhythmias. Women with NYHA class > III have a relatively poor prognosis during pregnancy [ 13 ]. Of note, one uncorrected patient with NYHA class IV was the only patient with limited left ventricular systolic function and pulmonary arterial hypertension. The pregnancy was terminated in that patient at 27 + 6 weeks due to severe ventricular failure. The data obtained from the current study collectively implied that the high degree of right ventricular dilation and high NYHA classification are the most relevant factors for negative outcomes.

It has been demonstrated that women at childbearing age with surgical repair for TOF have lower pregnancy-related risks, and the changes in ventricular dimensions and NYHA class are consistent with normal pregnancy adaptation [ 14 ]. In the literature, it has been demonstrated that pregnant patients even with corrected TOF still have a higher risk and poorer outcome than otherwise healthy women. The increased risk may be attributed to the extra haemodynamic burden and exacerbation of residual cardiovascular lesions, or recurrence of right ventricular outflow obstruction, right ventricular dilatation, pulmonary regurgitation, the right ventricular dilatation and failure, as well as atrial and ventricular arrhythmia [ 15 , 16 , 17 ]. In the current study, the residual shunt at VSD patches were present in two patients following operations and their NYHA class were grade III. In addition, 6 patients (31.58%) had pulmonary regurgitation and about two-thirds of patients had arrhythmia at different degrees. Our data support the notion that pregnant women with the surgically repaired TOF are still in high risk, depending on the degree of the cardiac functional adaption during pregnancy.

Regarding the functional adaption of pregnant women with corrected TOF, Egidy et al monitored the quantitative volumetric changes, and concluded that that those women with successful pregnancy appeared to experience an accelerated rate of right ventricular remodeling (an increase in end-diastolic volume) [ 14 ]. In our study, the moderate or severe right ventricular dilation was found in all of uncorrected patients (100%), but in less than half of the patients in surgically repaired group (47.37%). In addition, the right ventricular hypertrophy and pulmonary valve stenosis were also been found in all of uncorrected patients (100%), but much less in the surgically repaired patients. The comparisons between the corrected and uncorrected patients revealed that the risk of the women with corrected TOF might be divided to two groups, high-risk and low-risk patient groups according to the degree of right ventricular dilation/hypertrophy and the pulmonary valve stenosis. Further study with a large number of cases that are sufficient to stratify data for high-risk and low-risk patients is necessary to define their actual prognostic value.

Maternal condition and mortality has significant effects on fetal outcome in CHD patients [ 18 ]. Recently, Ramage et al. reported 2114 births to women with adult congenital heart disease (ACHD) and suggested an association between several adverse neonatal/maternal outcomes and ACHD [ 19 ]. Their results showed that preterm births (< 37 weeks gestation) were 1.4 times higher in women with ACHD than those without ACHD. Women with ACHD also had a higher odd of having a preterm birth with a gestation less than 32 weeks. In addition, 12.8% women with ACHD and 8.7% of women without ACHD delivered an SGA infant. Consistent with the study conducted by Ramage, the complications were more frequently present in the uncorrected group in our study. Although all of the patients have survived, most all of them suffered cardiac and obstetric complications at varying degrees. The low maternal cardiac output, intrauterine growth restriction and the SGA may occur even after the surgical repair of TOF [ 20 ].

In consideration of high complication risks, woman with cardiac disease may be safer with a C-section delivery to avoid prolonged laboring time [ 21 ]. In this study, one patient with intrauterine fetal death undergone transvaginal complete curettage of uterine cavity, other uncorrected patients delivered by C-section successfully. Women at highest risk may benefit from preconception counseling and close clinical monitoring during pregnancy [ 19 ]. Careful interdisciplinary management among the cardiologist, obstetrician, anesthetist, and neonatologist, and detailed plans for delivery may improve the prognosis [ 22 , 23 ]. Although the differences of outcomes between 19 surgically repaired and 12 uncorrected cases were significant, the impact of other clinical parameters was not achieved in the current study due to the small number of cases in the subgroups. Further research with a large number of cases to determine the relationship between the outcomes and other clinical parameters may be helpful to establish more beneficial management regimen for TOF patients, especially for those without surgical repair. In addition, it will be beneficial to study the each pregnancy outcome of these TOF patients (with or without repair) in instead of per woman. However, it has not been performed in the current study due to the limitation to retrieve complete medical record for previous pregnancies and no record for new pregnancies in these patients.

In summary, TOF confers a high-risk of cardiac and obstetric complications for pregnant women, especially in those who did not received repair surgery. However, due to economic and regional constraints, patients who did not have a chance to receive timely surgical repair and got pregnancy were occasionally seen in our practice. In the past ten years, 12 such cases among 85,184 pregnancies (1.4 out of 10,000) was seen in our medical center. From our experience, these patients may still be able to have a successful pregnancy, but exclusively depending on professional care from multidisciplinary teams. In addition, while the exact implications of clinical parameters remain to be defined with a larger number of cases, the high degree of ventricular dilatation heart, high functional classifications, serious cardiac arrhythmias and pulmonary hypertension appeared to be associated with maternal and neonatal risks in the patients studied in the current investigation. Routine cardiac examination should be performed before pregnancy to exclude possible cardiac diseases and the cardiac surgery should be performed early. For pregnant women with TOF, close monitoring should be strengthened no matter whether they have received surgical repair or not. Decision for the mode of delivery should be individualized by weighing the risks and benefits in a given clinical situation [ 2 , 23 ].

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The raw data supporting this study can be requested via the corresponding author.

Abbreviations

Adult congenital heart disease

Atrial premature beat

Atrioventricular block

Blood pressure

• Congenital heart disease

Complete right bundle branch block

Caesarean section

Electrocardiography

Hospital day (total days in hospital)

Incomplete right bundle branch block

Low birth weight infant at term

Cardiac function grading (New York Heart Association)

Pulmonary arterial hypertension

Patent ductus arteriosus

Patent foramen ovale

Premature rupture of fetal membranes

Small for gestational age

Tetralogy of fallot

Uteroplacental doppler flow

Ventricular premature beat

Ventricular septal defect

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Acknowledgements

We thank Drs. Hannah H Chen and John J Xin (Tufts University School of Medicine, Tufts Medical Center) for discussing and editing manuscript. We feel grateful for the doctors and staff who have been involved in this work.

This study was supported by the Academic and Technical Leader’s Foundation of Sichuan Province (No.2017–919-25), the Science Foundation of Sichuan Province (2018FZ0041) and the Health and Family Planning Commission Foundation of Sichuan Province (17PJ236). With the support of the first two funds (No.2017–919-25 and 2018FZ0041), we were able to design the study, complete the data collection and analysis; with the third fund (17PJ236), we were able to interpret the results, prepare the manuscript. The publishing fee will be also covered by the above fund.

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Kana Wang, Xiaodong Wang, Haiyan Yu & Xinghui Liu

Key Laboratory of Birth Defects and Related Diseases of Women and Children, Sichuan University, Ministry of Education, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, Sichuan, China

School of Public Heath, Chengdu Medical College, Chengdu, China

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WKN carried out the retrospective review of all cases, participated in the writing and organization of the manuscript. XJG participated in the study’s design and the analysis of cases. HYY and XDW conceived of the whole study, and carried out the study design and correction of the manuscript. LXH participated in the study’s design and coordination and helped to draft the manuscript. All authors read and approved the final manuscript.

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Wang, K., Xin, J., Wang, X. et al. Pregnancy outcomes among 31 patients with tetralogy of Fallot, a retrospective study. BMC Pregnancy Childbirth 19 , 486 (2019). https://doi.org/10.1186/s12884-019-2630-y

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• Tetralogy of Fallot (TOF)
• Surgical repair
• Uncorrected
• Pregnancy outcome

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Repaired Tetralogy of Fallot: Have We Understood the Right Timing of PVR?

Affiliations.

• 1 Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
• 2 Clinical Pathways and Epidemiology Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
• 3 Division of Cardiology and CardioLab, Department of Clinical Sciences and Translational Medicine, University of Rome Tor Vergata, 00133 Rome, Italy.
• 4 Department of Cardiology, University of Palermo, 90133 Palermo, Italy.
• 5 Department of Experimental and Clinical Medicine, Magna Graecia University, 88100 Catanzaro, Italy.
• 6 Paediatric Cardiology and Congenital Heart Disease, University of Padua and Pediatric Research Institute (IRP), Città Della Speranza, 35127 Padua, Italy.
• 7 School of Medicine, University College of Dublin, Mater Misericordiae University Hospital, D07 R2WY Dublin, Ireland.
• 8 Dipartimento di Medicina Clinica e Sperimentale, Università di Messina, 98122 Messina, Italy.
• 9 Institute of Cardiovascular Sciences University College London, London WC1E 6BT, UK and Centre for Inherited Cardiovascular Diseases, Great Ormond Street Hospital, London WC1N 3JH, UK.
• 10 Adult Congenital Heart Disease Unit, AO Dei Colli, Monaldi Hospital, 80131 Naples, Italy.
• 11 Cardiovascular Sciences Department, AOU "Ospedali Riuniti", 60126 Ancona, Italy.
• PMID: 38731211
• PMCID: PMC11084704
• DOI: 10.3390/jcm13092682

Despite many advances in surgical repair during the past few decades, the majority of tetralogy of Fallot patients continue to experience residual hemodynamic and electrophysiological abnormalities. The actual issue, which has yet to be solved, is understanding how this disease evolves in each individual patient and, as a result, who is truly at risk of sudden death, as well as the proper timing of pulmonary valve replacement (PVR). Our responsibility should be to select the most appropriate time for each patient, going above and beyond imaging criteria used up to now to make such a clinically crucial decision. Despite several studies on timing, indications, procedures, and outcomes of PVR, there is still much uncertainty about whether PVR reduces arrhythmia burden or improves survival in these patients and how to appropriately manage this population. This review summarizes the most recent research on the evolution of repaired tetralogy of Fallot (from adolescence onwards) and risk factor variables that may favor or delay PVR.

Keywords: PVR; pulmonary insufficiency; tetralogy of Fallot.

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About Tetralogy of Fallot

• Tetralogy of Fallot (pronounced te-tral-uh-jee of Fal-oh) is a congenital heart defect. Congenital means present at birth.
• Tetralogy of Fallot occurs when a when a baby’s heart does not form correctly during pregnancy.
• People with this condition should schedule routine checkups with a heart doctor to stay as healthy as possible.

Tetralogy of Fallot is made up of the following four defects of the heart and its blood vessels:

• A hole in the wall between the two lower chambers, or ventricles, of the heart. This condition also is called a Ventricular Septal Defect (VSD).
• A narrowing of the pulmonary valve and main pulmonary artery. This condition also is called pulmonary stenosis.
• The aortic valve, which leads to the aorta, is enlarged and receives blood from both ventricles. In a normal heart, it only receives blood from the left ventricle. In this defect, the aortic valve sits directly on top of the VSD.
• The muscular wall of the lower right chamber of the heart (right ventricle) is thicker than normal. This also is called ventricular hypertrophy .

Because a baby with Tetralogy of Fallot may need surgery or other procedures soon after birth, this condition is considered a critical congenital heart defect (critical CHD).

About 1 in every 2,077 babies in the United States are born with Tetralogy of Fallot 1 . This means that about 1,768 babies are born with Tetralogy of Fallot each year.

Signs and symptoms

Tetralogy of Fallot can cause oxygen in the blood that flows to the rest of the body to be reduced. Babies born with Tetralogy of Fallot may have a bluish looking skin color, called cyanosis, because their blood doesn't carry enough oxygen. At birth, infants might not have blue-looking skin, but may later develop sudden episodes of bluish skin during crying or feeding. These episodes are called tet spells .

Infants with Tetralogy of Fallot or other conditions causing cyanosis can have problems including:

• A higher risk of getting an infection of the layers of the heart, called endocarditis
• A higher risk of having irregular heart rhythms, called arrhythmia
• Dizziness, fainting, or seizures, because of the low oxygen levels in their blood
• Delayed growth and development

Risk factors

The causes of Tetralogy of the Fallot among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. A combination of genes and other risk factors may increase the risk for Tetralogy of the Fallot. These factors can include things in a mother's environment, what she eats or drinks, or the medicines she uses.

Testing and diagnosis

Tetralogy of Fallot may be diagnosed during pregnancy or soon after a baby is born.

During pregnancy

During pregnancy, screening tests (prenatal tests) check for birth defects and other conditions. An ultrasound, a tool that creates pictures of the baby, may detect Tetralogy of the Fallot. If the health care provider suspects Tetralogy of the Fallot from the ultrasound, they can request a fetal echocardiogram to confirm the diagnosis. A fetal echocardiogram is a more detailed ultrasound of the baby's heart. This test can show problems with the structure of the heart and how the heart is working with this defect.

After the baby is born

Tetralogy of Fallot usually is diagnosed after a baby is born, often after the infant has an episode of turning blue during crying or feeding (a tet spell). Findings on a physical exam may make a healthcare provider suspect a baby has Tetralogy of Fallot, including bluish-looking skin or hearing a heart murmur. If these findings are present, the health care provider might request one or more tests to confirm the diagnosis. The most common test is an echocardiogram, which is an ultrasound of the heart.

Tetralogy of Fallot can also be detected with newborn pulse oximetry screening . Newborn screening using pulse oximetry can identify some infants with Tetralogy of Fallot before they show any symptoms.

Tetralogy of Fallot can be treated by surgery soon after the baby is born. During surgery, doctors widen or replace the pulmonary valve and enlarge the passage to the pulmonary artery. They also will place a patch over the VSD to close the hole between the two lower chambers of the heart. These actions will improve blood flow to the lungs and the rest of the body.

What to expect long-term

Most infants will live active, healthy lives after surgery. However, they will need routine checkups with a heart doctor to monitor their progress and check for other health conditions that might develop as they get older. As adults, they may need more surgery or medical care for other possible problems.

• Stallings EB, Isenburg JL, Rutkowski RE et al; for the National Birth Defects Prevention Network. National population-based estimates for major birth defects, 2016–2020. Birth Defects Res. 2024;116(1): https://doi.org/10.1002/bdr2.2301

Congenital Heart Defects (CHDs)

Congenital heart defects (CHDs) are conditions that are present at birth and can affect the structure of a baby’s heart and the way it works. They are the most common type of birth defect.

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Experiences with 180 Cases of Tetralogy of Fallot in Infants and Children *

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