Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

AIM: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec). METHODS: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study. RESULTS: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death. CONCLUSIONS: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage.

Long-term outcomes of tetralogy of Fallot repair: A 30-year experience with 960 patients.

OBJECTIVE: This study evaluates the long-term results of tetralogy of Fallot repair and assesses the risk factors for adverse outcomes. METHODS: This retrospective study included 960 patients who underwent transatrial transpulmonary tetralogy of Fallot repair between 1990 and 2020. RESULTS: A transannular patch was placed in 722 patients, and pulmonary valve preservation was achieved in 233 patients. The median age at tetralogy of Fallot repair was 9.4 (interquartile range, 6.2-14.2) months. The median follow-up duration was 10.6 (interquartile range, 5.4-16.3) years. There were 8 early deaths (0.8%) and 20 late deaths (2.1%). Genetic syndrome and pulmonary valve annulus Z score less than -3 were risk factors for mortality. The survival was 97.7% (95% confidence interval, 96.4-98.5) and 94.5% (95% confidence interval, 90.9-96.7) at 10 and 30 years, respectively. Freedom from any reoperation was 86.4% (95% confidence interval, 83.6-88.7) and 65.4% (95% confidence interval, 59.8-70.4) at 10 and 20 years, respectively. Postoperative right ventricular outflow tract peak gradient of 25 mm Hg or greater correlated with reoperation. Propensity score-matched analysis demonstrated that freedom from pulmonary valve replacement at 15 years was higher in the pulmonary valve preservation group compared with the transannular patch group (98.2% vs 78.4%, P = .004). Freedom from reoperation for right ventricular outflow tract obstruction at 15 years was lower in the pulmonary valve preservation group compared with the transannular patch group (P = .006). CONCLUSIONS: The long-term outcomes of tetralogy of Fallot repair are excellent. A postoperative right ventricular outflow tract peak gradient less than 25 mm Hg appears to be optimal to prevent reoperation. If the pulmonary valve size is suitable, pulmonary valve preservation reduces the risk of pulmonary valve replacement, yet increases the reoperation rate for right ventricular outflow tract obstruction.

Kawashima Procedure: The Impact of Age.

BACKGROUND: The results of the Kawashima operation are incompletely defined. Furthermore, optimal timing of Kawashima operation, an important consideration when managing desaturated young infants awaiting surgery, remains unclear. We reviewed our outcomes of Kawashima operation, with a focus on the impact of age. METHODS: We conducted a retrospective review of patients who underwent Kawashima operation from 1990 to 2020. RESULTS: Thirty patients underwent Kawashima operation at a median age of 11.7 months (interquartile range, 4.4-27.4). Left isomerism was present in 27 patients (90%). There were no early deaths. There were 2 patients (6.7%) who had Kawashima takedown, both attributed to hypoxia. Fontan completion was achieved in 25 patients (83%). Overall freedom from death and transplantation at 20 years was 67% (95% CI, 32%-87%). Freedom from death and transplantation at 10 years was 100% for children 3 to 6 months of age at the time of Kawashima, compared with 86.4% for older children (P = 1.0). However, children aged 3 to 6 months had longer ventilation time (P = .01), intensive care stays (P = .03), and hospital stays (P = .05). Freedom from death or transplantation at 20 years among those who had concomitant common atrioventricular valve repair was 33%, vs 79% for those who did not undergo common atrioventricular valve repair (P = .02). CONCLUSIONS: Kawashima operation can be performed with low operative risk and acceptable long-term outcomes. Performing Kawashima operation on a patient aged less than 6 months does not affect survival, but is associated with increased morbidity. Need for common atrioventricular valve operation carries significant risk of mortality, and more effective techniques for atrioventricular valve repair are required.

Surgery for paravalvular abscess in children.

Objective: To investigate the outcomes of surgery in children with paravalvular abscess at our institution. Methods: A retrospective review of all patients who underwent surgery for paravalvular abscess was performed. Results: Between 1989 and 2020, 30 patients underwent surgery for paravalvular abscess, of whom 5 (16.7%) had an intracardiac fistula and 6 (20.0%) had a pseudoaneurysm. Aortic annulus abscesses were most common, occurring in 23 patients (76.7%). Aortic root replacement was performed in 17 patients (56.7%), root reconstruction was performed in 4 (13.3%), and reconstruction of the central fibrous body was required in 5 (16.7%). Postoperatively, 7 patients (23.3%) required extracorporeal membrane oxygenation (ECMO) support, and 1 patient (3.3%) required permanent pacemaker insertion. There were 6 early deaths, 5 of whom were on ECMO, and no late deaths, with a 15-year survival of 79.7% (95% confidence interval [CI], 60.2%-90.3%). Deaths were from sudden cardiac arrest resulting in brain death in 3 patients, inability to wean from ECMO due to severe cardiac dysfunction in 2 patients, and cerebral mycotic aneurysm and hemorrhage in 1 patient. Freedom from reoperation was 40.0% (95% CI, 17.0%-62.3%) at 15 years Reoperation due to recurrence was rare, occurring in only 2 patients (6.7%). Streptococcus pneumoniae (hazard ratio [HR], 9.2; 95% CI, 1.6-51.7) and preoperative shock (HR, 6.4; 95% CI, 1.3-32.0) were associated with mortality. Central fibrous body reconstruction was associated with reoperation (HR, 4.4; 95% CI, 1.2-16.1). Conclusions: Although paravalvular abscess in children is associated with high early mortality, hospital survivors have good long-term survival. Reoperation is frequent, but is rarely due to recurrence of endocarditis.

Pulmonary Valve Replacement in Repaired Tetralogy of Fallot Through Limited Left Anterolateral Thoracotomy: An Alternative to Repeat Sternotomy.

We report two patients with repaired tetralogy of Fallot who underwent pulmonary valve replacement through a limited left anterolateral thoracotomy. We describe the technique in detail. Both patients were at risk of cardiac injury during repeat sternotomy. This approach reliably avoids the risk of cardiac injury during repeat sternotomy and appears to be safe, simple, and reproducible.

Arterial Switch Operation With Neoaortic Valve Replacement in a 13-Year Old Patient With Transposition of Great Arteries With Ventricular Septal Defect and Left Ventricular Outflow Tract Obstruction-A Case Report.

The surgical management of d-transposition of great arteries (d-TGAs) with ventricular septal defect (VSD) and left ventricular outflow tract obstruction (LVOTO) is ever evolving and still remains a challenge because of wide anatomic variability, age of presentation, surgical options available, and their variable long-term results in different series. We describe a patient with d-TGA, VSD, and LVOTO who presented to us at 13 years of age and underwent an arterial switch operation along with neoaortic valve replacement with a mechanical prosthesis. The postoperative course was uneventful, and at hospital discharge, the echocardiogram was satisfactory. We present the pros and cons of this hitherto undescribed treatment option.

Rare Combination of Pathologies Causing Mitral Stenosis and Mitral Regurgitation: A Case Report.

A supramitral ring is a rare cause of mitral stenosis, while an isolated mitral valve cleft is a rare cause of congenital mitral regurgitation. Fortunately, both the lesions are known to have good outcomes after surgical correction. Although each is known to be associated with a variety of other structural heart defects, their coexistence has not been reported previously. We report a case of a three- and half-year-old boy detected to have a rare combination of supramitral ring producing severe mitral stenosis with a coexisting cleft in the anterior leaflet of mitral valve causing severe mitral regurgitation. The patient underwent successful surgical repair with resolution of both mitral stenosis and regurgitation.

Left Superior Vena Cava Draining Into Left Atrium in Tetralogy of Fallot-Four Cases of a Rare Association.

Persistent left superior vena cava is a common congenital anomaly of the thoracic venous system. Left superior vena cava draining into left atrium is a malformation of sinus venosus and caval system. The anomaly may be a cause of unexplained hypoxia even in adults. It may give rise to various diagnostic and technical challenges during cardiac catheterization and open-heart surgery. It is often detected serendipitously during diagnostic workup. Isolated left superior vena cava opening into left atrium is very commonly associated with other congenital heart defects. But tetralogy of Fallot is very rarely associated with persistent left superior vena cava which drains into left atrium. We report four such cases who underwent surgical correction successfully.

Surgical Options for Uhl's Anomaly.

BACKGROUND: Uhl's anomaly is an extremely rare congenital heart defect characterized by a near total absence of the myocardium of the parietal wall of the right ventricle. Few reports of surgical management exist in literature. We present three patients with this anomaly who were managed with different surgical strategies. PATIENTS AND METHODS: Patient 1: This 43-month-old girl had maternal rubella syndrome with speech and hearing deficits and gross right heart failure. Diagnosis was made on echocardiography and magnetic resonance imaging. She underwent partial excision and plication of the right ventricular parietal wall and total cavopulmonary connection. Patient 2: This 19-month-old boy presented with progressive cyanosis and features of right heart failure. Diagnosis was confirmed on echocardiography. He underwent right ventricular exclusion by tricuspid valve closure and free wall plication followed by a bidirectional Glenn procedure. Patient 3: This 21-year-old male presented with right heart failure and cyanosis. Diagnosis was established with transesophageal echocardiography and magnetic resonance imaging. As the hemodynamics were not suitable for a Fontan conversion, a one and a half ventricle repair was done along with plication of the right ventricular free wall and tricuspid valve annuloplasty. RESULTS: All three patients were extubated within 24 hours. Patients 2 and 3 were discharged relatively uneventfully, whereas patient 1 had a more prolonged stay due to transient hepatic failure. All were symptomatically and clinically improved on short-term follow-up. CONCLUSION: Uhl's anomaly is an extremely rare condition with varied clinical presentation. Surgical exclusion of the right ventricle yields gratifying results; however, surgical technique has to be adapted to the individual patient.