Peritoneal dialysis in an adult patient with tetralogy of Fallot diagnosed with incomplete Alagille syndrome.

BACKGROUND: Alagille syndrome is an autosomal dominant disorder usually caused by pathogenic variants of the JAG1 gene. In the past, cholestasis was a condition sine qua non for diagnosis of the syndrome. However, recent advancements in genetic testing have revealed that clinical presentations vary from lack of symptoms, to multiorgan involvement. Tetralogy of Fallot, the most frequent complex congenital heart defect in Alagille Syndrome, very rarely leads to renal failure requiring dialysis - there are only single reports of such cases in the literature, with none of them in Alagille Syndrome. CASE PRESENTATION: A 41-year-old woman suffering from cyanosis, dyspnea and plethora was admitted to the hospital. The patient suffered from chronic kidney disease and tetralogy of Fallot and had been treated palliatively with Blalock-Taussig shunts in the past; at admission, only minimal flow through the left shunt was preserved. These symptoms, together with impaired mental status and dysmorphic facial features, led to extensive clinical and genetic testing including whole exome sequencing. A previously unknown missense variant c.587G > A within the JAG1 gene was identified. As there were no signs of cholestasis, and subclinical liver involvement was only suggested by elevated alkaline phosphatase levels, the patient was diagnosed with incomplete Alagille Syndrome. End-stage renal disease required introduction of renal replacement therapy. Continuous ambulatory peritoneal dialysis was chosen and the patient's quality of life significantly increased. However, after refusal of further treatment, the patient died at the age of 45. CONCLUSIONS: Tetralogy of Fallot should always urge clinicians to evaluate for Alagille Syndrome and offer patients early nephrological care. Although tetralogy of Fallot rarely leads to end-stage renal disease requiring dialysis, if treated palliatively and combined with renal dysplasia (typical of Alagille Syndrome), it can result in severe renal failure as in the presented case. There is no standard treatment for such cases, but based on our experience, peritoneal dialysis is worth consideration. Finally, clinical criteria for the diagnosis of Alagille Syndrome require revision. Previously, diagnosis was based on cholestasis - however, cardiovascular anomalies are found to be more prevalent. Furthermore, the criteria do not include renal impairment, which is also common.

Short to medium term outcomes of right ventricular outflow tract stenting as initial palliation for symptomatic infants with complete atrioventricular septal defect with associated tetralogy of Fallot.

OBJECTIVES: To assess the impact of right ventricular outflow tract (RVOT) stenting as the primary palliation in infants with complete atrioventricular septal defect with associated tetralogy of Fallot (cAVSD/TOF). BACKGROUND: Historically, palliation of symptomatic patients with cAVSD/TOF has been achieved through surgical systemic to pulmonary artery shunting. More recently RVOT stenting has evolved as an acceptable alternative in patients with tetralogy of Fallot. METHODS: Retrospective review of all patients with cAVSD/TOF who underwent RVOT stenting as palliation over a 13-year period from two large tertiary referral centers. RESULTS: Twenty-six patients underwent RVOT stenting at a median age of 57 days (interquartile range [IQR] 25.5-106.5). Median weight for stent deployment was 3.7 kg (IQR 2.91-5.5 kg). RVOT stenting improved oxygen saturations from a median of 72% (IQR 70-76%) to 90% (IQR 84-92%), p < .001. There was a significant increase in the median Z-score for both branch pulmonary arteries at median follow-up of 255 days (IQR 60-455). Eight patients required RVOT stent balloon dilatations and 8 patients required re-stenting for progressive desaturation. The median duration between reinterventions was 122 days (IQR 53-294 days). Four patients died during the follow-up period. No deaths resulted from the initial intervention. To date, definitive surgical intervention was achieved in 19 patients (biventricular repair n = 15) at a median age of 369 days (IQR 223-546 days). CONCLUSION: RVOT stenting in cAVSD/TOF is a safe and effective palliative procedure in symptomatic infants, promoting pulmonary artery growth and improving oxygen saturations.

Early palliative balloon pulmonary valvuloplasty in neonates and young infants with tetralogy of Fallot.

We aim to clarify the efficacy of early palliative balloon pulmonary valvuloplasty (BPV) in neonates and young infants (< 60 days) with tetralogy of Fallot (TOF). We performed palliative BPV in 31 subjects, regardless of the presence of cyanosis, with Z score of the pulmonary valve diameter (PVD) less than - 2.00. Primary and secondary endpoints were to avoid early surgical interventions for subjects within 6 months of age and to undergo the pulmonary valve-sparing procedure at corrective surgery, respectively. We studied factors associated with these outcomes among them. BPV was performed at 19 days (14-33) of age and with a weight of 3.34 kg (3.02-3.65). Systemic oxygen saturation, Z score of the PVD, and pulmonary arterial index (PAI) were 87% (81-91), - 3.56 (- 4.15 to - 2.62), and 128 mm2/m2 (102-157), respectively. There were 16 and 13 subjects who avoided early surgical interventions and transannular repair, respectively. At the primary endpoint, there was no significant difference in age, weight, systemic oxygen saturation, and Z score of the PVD and PAI between the groups. However, there was a significant difference in the infundibular morphology (severe: mild-to-moderate, 8:8 vs 13:2, P = 0.029) between the groups. We performed prophylactic BPV within 30 days after birth in 7 acyanotic TOF patients with severe infundibular obstruction, among whom 5 avoided early surgical intervention. At the secondary endpoint, there were no significant difference in weight, systemic oxygen saturation, but in sex, age at BPV, and Z score of the PVD. Early palliative BPV prevented early surgical intervention in half of the neonates and young infants with TOF, which depended upon the degree of infundibular obstruction. However, early palliative BPV did not contribute to avoid transanular patch right-ventricular outflow repair among them.

Tetralogy of Fallot with concurrent patent foramen ovale and tricuspid valve dysplasia in a dog.

The echocardiographic findings of a young Pomeranian-cross dog with tetralogy of Fallot, patent foramen ovale, and tricuspid valve dysplasia are described. Ongoing medical management of hypoxemia and erythrocytosis was carried out and the dog survived to 2 years of age. Treatment options for tetralogy of Fallot are discussed.

Tétralogie de Fallot avec persistance du foramen ovale et dysplasie de la valve tricuspide concomitante chez un chien. Les constatations écho-cardiographiques chez un chien Poméranien de race croisée atteint de la tétralogie de Fallot, de la persistance du foramen ovale et d'une dysplasie de la valve tricuspide sont décrites. Une gestion médicale constante de l'hypoxémie et de l'érythrocytose a été réalisée et le chien a survécu jusqu'à l'âge de 2 ans. Les options de traitement pour la tétralogie de Fallot sont discutées.(Traduit par Isabelle Vallières).

Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot.

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.

Stenting of the Right Ventricular Outflow Tract Promotes Better Pulmonary Arterial Growth Compared With Modified Blalock-Taussig Shunt Palliation in Tetralogy of Fallot-Type Lesions.

OBJECTIVES: This study sought to compare pulmonary arterial (PA) growth during palliation after right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) in patients coming forward for complete repair of tetralogy of Fallot-type lesions. BACKGROUND: RVOT stenting is a recent alternative to mBTS in the initial palliation of selected patients with Fallot-type lesions. METHODS: This was a retrospective, single-center study of nonrandomized, consecutive palliated Fallot patients over a 10-year period. Differential left PA (LPA) and right PA (RPA) growth was assessed by serial echocardiograms in 67 patients after mBTS (n = 28) or RVOT stent (n = 39). Statistical data analysis was performed using mixed model analysis. RESULTS: RPA z-scores in the mBTS group improved from median -2.41 (interquartile range [IQR]: -2.97 to -1.32) to -1.13 (IQR: -1.68 to -0.59). LPA z-scores improved from -1.89 (IQR: -2.33 to -1.12) to -0.32 (IQR: -0.88 to -0.05). In the RVOT stenting group RPA z-scores improved from -2.28 (IQR: -3.28 to -1.82) to -0.72 (IQR: -1.27 to +0.48), and LPA z-scores from -2.08 (IQR: -2.90 to -0.61) to -0.05 (IQR: -0.88 to +0.48). Mixed model analysis showed significantly better RPA and LPA growth after RVOT stenting. The benefit of RVOT stenting versus mBTS was 0.599 z-scores for the LPA and 0.749 z-scores for the RPA. Rise in oxygen saturations was greater with RVOT stenting (p = 0.012). Median time to complete repair was shorter in the RVOT stent group (227 [142 to 328] days) compared with the mBTS group (439 [300 to 529] days; p < 0.0003). CONCLUSIONS: RVOT stenting promotes better pulmonary arterial growth and oxygen saturations compared with mBTS in the initial palliation of Fallot-type lesions.

Effects of Angle Correction Angioplasty for Pulmonary Artery Stenosis With Tetralogy of Fallot.

BACKGROUND: Left pulmonary artery (LPA) stenosis with acute angulation is a common cause of reoperation after tetralogy of Fallot repair. We therefore investigated surgical outcomes of acute-angle correction angioplasty. METHODS: Between 2005 and 2012, 53 patients underwent operation for LPA stenosis as a concurrent procedure. We divided the patients into two groups according to the LPA ostium angulation: group I (acute angle, acute-angle correction angioplasty, n = 29) and group II (obtuse angle, conventional patch angioplasty, n = 24), encompassing subgroup type I (focal stenosis) and type II (diffuse stenosis). The changes in the z score of LPA and lung perfusion rates were monitored using computed tomography and lung perfusion scans. RESULTS: The z scores of LPA ostium and hilum were improved at 4.9 mean years of follow-up in group I (p = 0.002, p = 0.041). At the most recent follow-up, there were no differences in the LPA z score (p = 0.177; n = 16 in group I; n = 13 in group II) or left lung perfusion rates (group I: 39.5% ± 9.6%, n = 26; group II: 36.8% ± 11.2%, n = 18, p = 0.418) between the two groups. In group I, the ostial z scores and lung perfusion rates in patients with type II (-4.1 ± 3.1, 25.4%) were lower than patients with type I (-1.6 ± 2.5, 41.0%) at the most recent follow-up (p = 0.069, p = 0.001, respectively). The reoperation or reintervention rate was higher in patients with type II than in patients with type I in group I (p = 0.022). CONCLUSIONS: Acute-angle correction angioplasty was associated with similar LPA ostium z scores and left lung perfusion rates in the two groups. Diffuse LPA stenosis compromised left lung perfusion and increased reoperation or reintervention rate.

Effects of Balloon Pulmonary Valvuloplasty as Preoperative Palliation for Tetralogy of Fallot.

OBJECTIVES: We investigated the effect of balloon pulmonary valvuloplasty (BPV) as a palliative procedure for patients with tetralogy of Fallot (TOF). METHODS: This was a retrospective single-center study conducted between 2008 and 2014. We classified patients into three groups according to palliation: treatment with BPV (group I), with a Blalock-Taussig shunt (group II), and with infundibulectomy (group III). The growth of the pulmonary valve (PV) annulus and need for transannular patching (TAP) during total correction were compared between groups. RESULTS: Forty-eight patients were enrolled: 31, 10, and 7 in groups I, II, and III, respectively. The mean ages at palliation were 26.3 ± 23.6, 21.5 ± 16.4, and 15.2 ± 4.2 days in groups I, II, and III, respectively (P = .867). The success rate of BPV was 90.3%. The initial PV z-score before palliation did not differ between groups I, II, and III (-3.14 ± 1.44, -3.84 ± 1.47, and -3.71 ± 1.68; P = .494); the preoperative PV z-score before total correction was larger in group I than in group II (-1.19 ± 1.14 and -3.05 ± 1.19; P = .001), and also larger in group III than in group II (group III: -1.10 ± 0.90; P = .003). The change in pulse oxygen saturation differed significantly between the three groups (P = .031), particularly between groups I and II (P = .010), and the change in PV z-score was greater in groups I and III than in group II (P < .001). TAP during total correction was performed in 29.0%, 90.0%, and 16.7% of groups I, II, and III, respectively (P = .001; group I vs. II, P = .001; group II vs. III, P = .008). CONCLUSION: BPV was an effective and safe palliation that increased pulmonary blood flow and promoted growth of the PV annulus in selected symptomatic newborns with TOF.

Contemporary Hospitalization Rate Among Adults With Complex Congenital Heart Disease.

BACKGROUND: Adults with congenital heart disease are hospitalized at increasing rates in the Western world. Identification of rates of and risk factors for hospitalization is essential for research and improving patient outcomes. METHODS: We conducted a single-center retrospective analysis of patients with a primary diagnosis of tetralogy of Fallot (TOF), transposition of the great arteries (both d- and l-transposition of the great arteries [TGAs]), or single ventricle (SV). We investigated the rates of mortality, annual hospitalization, and patient-specific risk factors for unplanned cardiac hospitalization. RESULTS: Adult patients with complex congenital heart disease are hospitalized at a rate four to eight times greater than the general US population (P < .001). In addition, there are significant differences between the rates of hospitalization in TOF and TGA (0.39 and 0.41 hospitalizations per patient-year, respectively) and SV (0.72 hospitalizations per patient-year). The majority of excess hospitalizations in the study group were due to cardiac disease (P < .001 for all three groups). Risk factors for unplanned cardiac hospitalization in TOF included pulmonary atresia, depressed left ventricular and right ventricular ejection fraction, and smoking; in TGA, they included Ebstein malformation, surgeries other than primary repair, noncardiac diagnoses, atrial arrhythmias, atrioventricular nodal block, left ventricular ejection fraction, and smoking; and in SV, they included atrial arrhythmias and cyanosis. CONCLUSIONS: Patients born with complex congenital heart disease are hospitalized far more frequently than the general US population, primarily for cardiac-related illness. Future research should focus on confirming the present findings and on identification of strategies to improve outcomes in this growing group of patients.

Palliative balloon dilation of pulmonic stenosis in a dog with tetralogy of Fallot.

A 6-month-old Beagle with tetralogy of Fallot underwent balloon valvuloplasty of the pulmonary valve. Balloon valvuloplasty was successful and resulted in palliation of clinical signs and an improved quality of life for approximately 9 months. After 9 months, the dog became symptomatic and a modified Blalock-Taussig shunt procedure was successfully performed. Based on this report, balloon valvuloplasty in dogs with tetralogy of Fallot appears to be a feasible technique that may result in improvement of clinical signs. In addition, it may allow for the delay of the more invasive surgical palliation and provide time for weight gain and development of the pulmonary vascular bed for greater ease of surgical shunt creation.

Use of Pulmonary Hypertension Medications in Patients with Tetralogy of Fallot with Pulmonary Atresia and Multiple Aortopulmonary Collaterals.

Tetralogy of Fallot (TOF) with pulmonary atresia (PA) and multiple aortopulmonary collaterals (MAPCAs) is a rare and severe form of congenital heart disease with poor prognosis. Aortopulmonary collaterals expose pulmonary arterioles to systemic pressure resulting in pulmonary hypertension (PH). To date, reports regarding the role of PH medications in this population are sparse. The objective of this study was to assess the effect of PH medications in patients with TOF, PA and MAPCAs or similar anatomy, with emphasis on symptoms, echocardiography and invasive hemodynamics. A retrospective review was performed for patients at a single tertiary care pediatric center. Twelve of 66 patients were treated with PH medications (18 %), and eight of these patients had adequate follow-up for further analysis. Median age at last follow-up was 6 years (range 1.4-21 years). Median length of therapy with PH medication was 4 years (range 0.3-17 years). PH medications included sildenafil, bosentan, ambrisentan, inhaled treprostinil and prostacyclin infusion. PH therapy was associated with improvement in symptoms in all patients and improvement in PH by hemodynamic measures in the majority of patients. All patients underwent at least one cardiac intervention by catheterization or surgery while taking PH medication. Two patients died from non-PH-related causes. The remaining six patients are alive and remain on PH medication. This review indicates that PH medications are well tolerated by this patient group and provide symptomatic improvement. Further studies are required to determine whether PH medications provide long-term survival benefit for patients with complex congenital heart disease.

The role of curcumin as an inhibitor of oxidative stress caused by ischaemia re-perfusion injury in tetralogy of Fallot patients undergoing corrective surgery.

BACKGROUND: Cardiopulmonary bypass during tetralogy of Fallot corrective surgery is associated with oxidative stress, and contributes to peri-operative problems. Curcumin has been known as a potent scavenger of reactive oxygen species, which enhances the activity of antioxidants and suppresses phosphorylation of transcription factors involved in inflamation and apoptosis. OBJECTIVES: To evaluate the effects of curcumin as an antioxidant by evaluating the concentrations of malondialdehyde and glutathione, activity of nuclear factor-kappa B, c-Jun N-terminal kinase, caspase-3, and post-operative clinical outcomes. METHODS: Tetralogy of Fallot patients for corrective surgery were randomised to receive curcumin (45 mg/day) or placebo orally for 14 days before surgery. Malondialdehyde and glutathione concentrations were evaluated during the pre-ischaemia, ischaemia, re-perfusion phases, and 6 hours after aortic clamping-off. Nuclear factor-kappa B, c-Jun N-terminal kinase, and caspase-3, taken from the infundibulum, were assessed during the pre-ischaemia, ischaemia, and re-perfusion phases. Haemodynamic parameters were monitored until day 5 after surgery. RESULTS: In all the observation phases, malondialdehyde and glutathione concentrations were similar between groups. There was no significant difference in nuclear factor-kappa B activity between the groups for three observations; however, in the curcumin group, c-Jun N-terminal kinase significantly decreased from the pre-ischaemia to the re-perfusion phases, and caspase-3 expression was lower in the ischaemia phase. Patients in the curcumin group had lower temperature and better ventricular functions, but no significant differences were found in mechanical ventilation day or length of hospital stay in the two groups. CONCLUSION: Cardioprotective effects of curcumin may include inhibition of the c-Jun N-terminal kinase pathway and caspase-3 in cardiomyocytes, particularly in the ischaemia phase.

Extracorporeal membrane oxygenation in children with heart disease and down syndrome: a multicenter analysis.

The data on the outcomes of children with heart disease and Down syndrome receiving extracorporeal membrane oxygenation (ECMO) for cardiac or respiratory failure are limited. This study aimed to evaluate morbidity and mortality associated with ECMO in children with Down syndrome and heart disease. Children younger than 18 years undergoing heart surgery and ECMO reported in the Extracorporeal Life Support Organization (ELSO) registry (1998-2011) were included in the study. The registry was queried for the following five heart defects: common atrioventricular (AV) canal, tetralogy of Fallot, truncus arteriosus, transposition of great vessels, and interrupted aortic arch. Data collection included patient characteristics, ECMO characteristics, and outcomes. The outcomes evaluated included mortality, ECMO duration, and length of hospital stay for patients with Down syndrome and those with no Down syndrome. The study enrolled 2,815 patients qualified for inclusion. Of these patients, 121 had Down syndrome, whereas 2,694 had no genetic syndrome and were included in the control group. The median age of the patients was 45 days (interquartile range [IQR] 9-192 days), and the median weight was 3.8 kg (IQR 3.0-6.1 kg). The most common cardiac defects in Down syndrome group were common AV canal (63 %) and tetralogy of Fallot (40 %). The Down syndrome group included older patients with greater body weight than the control group. The mortality rate was lower in the Down syndrome group than in the control group (44 vs. 56 %; p = 0.01). The duration of ECMO and length of hospital stay were similar in the two groups. The findings showed that ECMO can be used for children with heart disease and Down syndrome with good results. The outcomes were comparable between the children with Down syndrome and the children without Down syndrome.

Hybrid perventricular pulmonary valve perforation and right ventricular outflow stent placement: a case report of a premature, 1.3-kg neonate with tetralogy of Fallot and pulmonary atresia.

Premature infants with very low birth weight with tetralogy of Fallot and pulmonary atresia (TOF/PA) have been shown to have poor surgical outcomes. Palliating these patients by stenting the right ventricular outflow tract (RVOT) has been shown to be safe and effective. In patients with very low birth weight, a hybrid perventricular approach offers the ability to perform pulmonary valve perforation and RVOT stent placement while avoiding cardiopulmonary bypass and femoral vessel complications in the neonatal period. We present a hybrid perventricular treatment of a patient weighing 1.3 kg with TOF/PA.

Denervação simpática cirúrgica para o controle de tempestade arrítmica e choques repetidos de desfibrilador implantável em criança com síndrome do QT longo congênito / Sympathetic denervation surgery to control of arrhythmic storm and repeated chocks with the implantable defibrillator in children with congenital long QT syndrome

Relata-se o caso de uma criança com síndrome do QT longo congênito e Tetralogia de Fallot, submetidaa denervação simpática por videotoracoscopia, para controle de tempestades arrítmicas e diminuição do númerode choques do cardioversor-desfibrilador implantável...

We report the case of a child with congenital long QT syndrome and Tetralogy of Fallot submittedto sympathetic denervation by videolaparoscopy to control arrhythmic storms and decrease the number ofimplantable cardioversor-defibrillator chocks...

Stenting of the right ventricular outflow tract in symptomatic neonatal tetralogy of Fallot.

There is ongoing debate regarding the initial management of symptomatic neonates with tetralogy of Fallot. Although neonatal repair can be performed with low mortality, it is associated with increased morbidity and long-term impact on right ventricular performance. Traditionally, the modified Blalock-Taussig shunt remains the palliative procedure of choice. Differential pulmonary artery flow may occur and subsequently result in underdevelopment and distortion of pulmonary vessels. Transcatheter therapy was previously limited to balloon valvulotomy when the obstruction is predominantly at the pulmonary valve level. Stenting of the right ventricular outflow tract can enable adequate forward flow; however, pulmonary regurgitation may impact on right ventricular performance and cardiac output. Stenting of the right ventricular outflow tract with valve sparing placement of the stent thus treating the underlying pathophysiology of the hypercyanotic spells provides a safe and effective management strategy, improving arterial oxygen saturation, avoiding pulmonary regurgitation and encouraging pulmonary artery growth.

Hot topics in tetralogy of Fallot.

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart defect. We explore "hot topics" to highlight areas of emerging science for clinicians and scientists in moving toward a better understanding of the long-term management of patients with repaired TOF. From a genetic perspective, the etiology of TOF is multifactorial, with a familial recurrence risk of 3%. Cardiac magnetic resonance is the gold standard assessment tool based on its superior imaging of the right ventricular (RV) outflow tract, pulmonary arteries, aorta, and aortopulmonary collaterals, and on its ability to quantify biventricular size and function, pulmonary regurgitation (PR), and myocardial viability. Atrial re-entrant tachycardia will develop in more than 30% of patients, and high-grade ventricular arrhythmias will be seen in about 10% of patients. The overall incidence of sudden cardiac death is estimated at 0.2%/yr. Risk stratification, even with electrophysiologic testing and cardiac magnetic resonance, remains imperfect. Drug therapy has largely been abandoned, and defibrillator placement, despite its high risks for complications and inappropriate discharges, is often recommended for patients at higher risk. Definitive information about optimal surgical strategies for primary repair to preserve RV function, reduce arrhythmia, and optimize functional status is lacking. Post-operative lesions are often amenable to transcatheter intervention. In selected cases, PR may be treated with transcatheter valve insertion. Ongoing surveillance of RV function is a crucial component of clinical assessment. Except for resynchronization with biventricular pacing, no medical therapies have been shown to be effective after RV dysfunction occurs. In patients with significant PR with RV dilation, optimal timing of pulmonary valve replacement remains uncertain, although accepted criteria are emerging.

Transcatheter closure of postoperative residual ventricular septal defects using Amplatzer-type perimembranous VSD occluders.

OBJECTIVES: The reoperations of postoperative residual ventricular septal defects (VSDs) are associated with higher risks. Our aim is to assess the efficacy and safety of transcatheter closure of postoperative residual VSDs using perimembranous VSD occluders. METHODS: Twenty-one patients with residual VSDs underwent transcatheter closure in our center from January 2005 to January 2012. The study population consisted of 9 males and 12 females whose ages ranged from 1.9 to 54 years (median age, 8.7 years). Eighteen cases had perimembranous VSD repair previously, 3 cases had tetralogy of Fallot surgical treatment. All patients had signs of left ventricle volume overload (Qp/Qs ≥ 1.5). Two types of perimembranous VSD occluders, symmetric and asymmetric, were used in 14 and 7 cases, respectively. The diameter of residual VSDs ranged from 4-16 mm (mean, 7.8 mm). The waist size of occluders ranged from 6-18 mm. RESULTS: There were 0 deaths and 1 serious adverse event. Intravascular hemolysis occurred in 1 patient (4.8%), lasted for 7 days, and recovered with therapy. A trivial intraprosthetic residual shunt was observed in 2 patients (9.5%) after the procedure and 1 patient (4.8%) at 6 months. Two patients (9.5%) had transient left anterior hemiblock and recovered within the first week after the procedure. At the latest follow-up, no atrioventricular block and new-onset aortic regurgitation occurred. CONCLUSIONS: Transcatheter closure is a feasible and safe management option for patients with postoperative residual VSDs and obviates the need for a second surgery and cardiopulmonary bypass.