Long-term outcomes of atrioventricular septal defect and single ventricle: A multicenter study.

OBJECTIVE: The study objective was to analyze survival and incidence of Fontan completion of patients with single-ventricle and concomitant unbalanced atrioventricular septal defect. METHODS: Data from 4 Dutch and 3 Belgian institutional databases were retrospectively collected. A total of 151 patients with single-ventricle atrioventricular septal defect were selected; 36 patients underwent an atrioventricular valve procedure (valve surgery group). End points were survival, incidence of Fontan completion, and freedom from atrioventricular valve reoperation. RESULTS: Median follow-up was 13.4 years. Cumulative survival was 71.2%, 70%, and 68.5% at 10, 15, and 20 years, respectively. An atrioventricular valve procedure was not a risk factor for mortality. Patients with moderate-severe or severe atrioventricular valve regurgitation at echocardiographic follow-up had a significantly worse 15-year survival (58.3%) compared with patients with no or mild regurgitation (89.2%) and patients with moderate regurgitation (88.6%) (P = .033). Cumulative incidence of Fontan completion was 56.5%, 71%, and 77.6% at 5, 10, and 15 years, respectively. An atrioventricular valve procedure was not associated with the incidence of Fontan completion. In the valve surgery group, freedom from atrioventricular valve reoperation was 85.7% at 1 year and 52.6% at 5 years. CONCLUSIONS: The long-term survival and incidence of Fontan completion in our study were better than previously described for patients with single-ventricle atrioventricular septal defect. A concomitant atrioventricular valve procedure did not increase the mortality rate or decrease the incidence of Fontan completion, whereas patients with moderate-severe or severe valve regurgitation at follow-up had a worse survival. Therefore, in patients with single-ventricle atrioventricular septal defect when atrioventricular valve regurgitation exceeds a moderate degree, the atrioventricular valve should be repaired.

Results of Coarctation Repair by Thoracotomy in Pediatric Patients: A Single Institution Experience.

BACKGROUND: Aortic coarctation is among the most common cardiovascular congenital abnormalities requiring repair after birth. Besides mortality, morbidity remains an important aspect. Accordingly, we reviewed our 20-year experience of aortic coarctation repair by thoracotomy, with emphasis on both short- and long-term outcomes. METHODS: From 1995 through 2014, 214 patients underwent coarctation repair via left thoracotomy. Associated arch lesions were distal arch hypoplasia (n = 117) or type A interrupted aortic arch (n = 6). Eighty-four patients had isolated coarctation (group 1), 66 associated ventricular septal defect (group 2), and 64 associated complex cardiac lesions (group 3). Median follow-up was 8.4 years. RESULTS: There was one (0.5%) procedure-related death. Nine (4.2%) patients died during index admission. In-hospital mortality was 0.7% in group 1 and 2 and 12.5% in group 3 (P < .001). No patient had paraplegia. Actuarial five-year survival was 97.5% in group 1, 94% group 2 and 66% in group 3. Recurrent coarctation developed in 29 patients, all but four (1.8%) successfully treated by balloon dilatation. Freedom from reintervention (dilatation or surgery) at five years was 86%. At hospital discharge, 28 (13.5%) patients were hypertensive. At follow-up, hypertension was present in 11 (5.3%) patients. CONCLUSIONS: Long-term results of aortic coarctation repair by thoracotomy are excellent, with percutaneous angioplasty being the procedure of choice for recurrences. Patient prognosis is dependent on associated cardiac malformations. In this study, the prevalence of late arterial hypertension was lower than previously reported.

The Pulmonary Autograft After the Ross Operation: Results of 25-Year Follow-Up in a Pediatric Cohort.

BACKGROUND: Progressive autograft dilation and need for later reoperation remain major concerns of the Ross procedure. The study investigates the clinical outcome after the Ross operation, including a longitudinal analysis of autograft dimensions over 25 years. METHODS: From November 1991 to April 2019, 137 patients underwent a Ross procedure at the University Hospitals of UCL (Université catholique de Louvain)-Brussels and Ghent. Inclusion criteria were less than or equal to 18 years of age and pulmonary autograft implantation by root replacement. Outcome focused on survival, reoperation rate, and autograft size evolution through linear mixed-model analysis. RESULTS: A Ross or Ross-Konno operation was performed in 110 (80%) and 27 (20%) patients at a median age of 10.4 (interquartile range [IQR], 4.7-14.3) years and 0.5 (IQR, 0.04-5.2) years, respectively. Overall 10-year and 20-year survival was 87% ± 3% and 85% ± 3%, respectively, but was 93% ± 3% for isolated Ross patients. Right ventricular outflow tract-conduit exchange was required in 20.3%, whereas autograft-related reoperation was performed in 14 (10.7%) patients at a median interval of 14 (IQR, 9-16) years, for aortic regurgitation (n = 2) and autograft dilation (n = 12). Autograft z-values increased significantly at the sinus and sinotubular junction (STJ) compared with the annulus (annulus = 0.05 ± 0.38/y, sinus = 0.14 ± 0.25/y, STJ = 0.17 ± 0.34/y; P = .015). The z-value slope for autograft dimensions was significantly steeper for Ross-Konno vs Ross patients (annulus: P = .029; sinus: P < .001; STJ: P = .012), and for children having aortic arch repair (annulus: P = .113, sinus: P = .038; STJ: P = .029). CONCLUSIONS: The Ross operation offers children requiring aortic valve replacement an excellent survival perspective, with an acceptable risk of autograft reoperation within the first 25 years. Contrary to the autograft annulus, dilation of the sinus and STJ size is of concern. Closer surveillance of autograft dimensions might be required in patients who underwent a Ross-Konno procedure or aortic arch reconstruction.

Thoracic duct-azygos vein anastomosis in an infant with superior vena cava syndrome and recurrent chylothorax.

In this study, we describe an intrathoracic microsurgical lymphatico-venous anastomosis as an alternative surgical technique for the treatment of refractory chylothorax in an infant. This procedure allowed us to restore enteral nutrition within days of surgery. At 3-year follow-up, there was no recurrence of pleural effusion.

Minimally invasive robotic excision of a cardiac septal neuroendocrine metastasis.

We present a rare case of a myocardial neuroendocrine metastasis in a 45-year-old male patient. The tumor was localized on the left side of the interventricular septum. Complete surgical excision of the tumor was successfully performed robotically  through a left atriotomy, using a transmitral approach. The patient's postoperative course was uneventful. A robotic mini-invasive approach is a safe and feasible alternative to conventional surgery and should be considered when the anatomy is suitable for a minimally invasive procedure.

Distal Aortic Arch Hypoplasia and Coarctation Repair: A Tailored Enlargement Technique.

BACKGROUND: Several techniques have been described to correct coarctation associated with distal arch hypoplasia. However, in neonates, residual gradients are frequently encountered and influence long-term outcome. We reviewed our experience with an alternative technique of repair combining carotid-subclavian angioplasty and extended end-to-end anastomosis. METHODS: From 1998 through 2014, 109 neonates (median age, 9 days) with coarctation and distal arch hypoplasia (n = 106) or type A interrupted aortic arch (n = 3) underwent repair using this technique. Thirty patients had isolated lesions (group 1), 44 associated ventricular septal defect (group 2), and 35 associated complex cardiac lesions (group 3). Median follow-up was 98 months. RESULTS: Repair was performed via left thoracotomy in 97%. There was one procedural-related death (0.9%) and overall five patients died during index admission (4.6%). Ten deaths were recorded at follow-up. Actuarial five-year survival was 86% (100% in group 1, 91% group 2, and 66% in group 3). Recurrent coarctation (clinical or invasive gradient >20 mm Hg) developed in 15 patients, all but 2 successfully treated by balloon dilatation. Freedom from any reintervention (dilatation or surgery) at five years was 86%. Only two patients were on antihypertensive drugs at last follow-up. CONCLUSIONS: This combined technique to correct distal arch hypoplasia and isthmic coarctation results in low mortality and acceptable recurrence rate. It preserves the left subclavian artery and allows enlargement of the distal arch diameter. Late outcome is excellent with very low prevalence of late arterial hypertension.

Recent Experience and Follow-Up After Surgical Closure of Secundum Atrial Septal Defect in 120 Children.

While percutaneous catheter closure proves an effective treatment for secundum atrial septal defect (ASD2), some child patients require surgical closure. We assessed the risks associated with isolated surgical ASD2 closure by reviewing the outcomes of 120 children operated on between 1999 and 2011 (mean age 4.6 ± 3.9 years, mean weight 17 ± 12 kg). Direct sutures were performed in 4% and patch closures in 96%. The mean cardiopulmonary bypass duration was 38 ± 14 min, aortic cross-clamp time 19 ± 9 min, intensive care unit length of stay 1.6 ± 1.1 days, hospital stay 11.2 ± 5.1 days. There were no complications in 60 patients (50%) and major complications in 8 (6.7%), with 1 patient (0.8%) dying of pneumonia-induced sepsis, 2 (1.7%) requiring revision surgery, 3 (2.5%) requiring invasive treatment (2 pericardial drainage, 1 successful resuscitation), and 2 (1.7%) presenting thromboembolisms (1 cerebral stroke, 1 cardiac thrombus). In hospital minor complications occurred in 22 patients: 17 pericardial effusions (15%), 15 infections requiring treatment (12.5%), 1 sternal instability (0.8%), 4 anemias requiring transfusion (3.3%), 7 pulmonary atelectasis (6%), and 2 post-extubation glottis edema (1.7%). At early outpatient follow-up, complications occurred in 21 patients: 16 (13.3%) pericardial effusions, 4 (3.3%) infections requiring treatment, and 3 (2.5%) keloid scarring. No complications occurred during long-term follow-up. In line with published data, mortality was low (0.8%), yet major complications (6.7%) were more common in these cases than those following percutaneous ASD2 closure. Minor complications were frequent (43%) with no long-term sequelae.

Heart transplantation using a donor with partial anomalous pulmonary venous connection and atrial septal defect.

Over the last decade, the shortage of donors has led to increased waiting time prior to transplantation and its related mortality. Therefore, extended criteria for donor hearts have been proposed. In this report, we describe a successful transplantation despite a diagnosis of partial abnormal pulmonary venous return associated with an atrial septal defect sinus venosus and persisting left-sided superior vena cava. Knowledge in congenital cardiac disease can broaden the definition of 'marginal' donor hearts and allow their use without increasing the risk of transplantation.