Can Yerebakan

Hybrid therapy for hypoplastic left heart syndrome: Myth, alternative, or standard?

OBJECTIVE This retrospective study presents our operative results, mortality, and morbidity with regard to pulmonary artery growth and reinterventions on the pulmonary artery and aortic arch, including key features of our institutional standards for the 3-stage hybrid palliation of patients with hypoplastic left heart syndrome. METHODS Between June 1998 and February 2015, 182 patients with hypoplastic left heart structures underwent the Giessen hybrid stage I procedure. Among these, 126 patients with hypoplastic left heart syndrome who received a univentricular palliation or heart transplantation were included in the main analysis. Median age and body weight of patients at hybrid stage I were 6 days (0-237) and 3.2 kg (1.2-7), respectively. Comprehensive stage II operation was performed at 4.5 months (2.9-39.5), and Fontan completion was established at 33.7 months (21.1-108.2). Operative and interstage mortality, morbidity, growth and reinterventions on the pulmonary arteries, and long-term operative results of the aortic arch reconstruction were assessed. RESULTS Median follow-up time after Giessen hybrid stage I palliation was 4.6 years (0-16.8). Operative mortality at hybrid stage I, comprehensive stage II, and Fontan completion was 2.5%, 4.9%, and 0%, respectively. Cumulative interstage mortality was 14.2%. At 10 years, the probability of survival is 77.8%. Body weight (<2.5 kg) and aortic atresia had no significant impact on survival. McGoon ratio did not differ at comprehensive stage II and Fontan completion (P = .991). Freedom from pulmonary artery intervention was estimated to be 32.2% at 10 years. Aortic arch reinterventions were needed in 16.7% of patients; 2 reoperations on the aortic arch were necessary. CONCLUSIONS In view of the early results and long-term outcome, the hybrid approach has become an alternative to the conventional strategy to treat neonates with hypoplastic left heart syndrome and variants. Further refinements are warranted to decrease patient morbidity.

Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes.

Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH.

Transcatheter interatrial communications for the treatment of left heart disease: Application in the pediatric population

Reduction of left atrial pressure by transcatheter creation of an interatrial communication in patients who present with left ventricular (LV) failure has recently been introduced. Such an approach allows left-to-right shunting at the atrial level aiming to reduce pulmonary congestion. Although “hard” outcome parameters were not assessed, a marked improvement in functional status and cardiac output, accompanied by a significant reduction in pulmonary capillary wedge pressure (PCWP), was observed. The reported strategy can be extended to other forms of LV diseases. On the basis of our long-term experience with manipulation of the interatrial septum in neonates born with various forms of hypoplastic left heart to decompress the left atrium, we expanded this knowledge to children and young adults with restrictive cardiomyopathy (RCM) and to those with dilated cardiomyopathy (DCM). We report the use of transcatheter atrial septostomy in 5 patients, aged 6.6 years (range, 0.9–32.0 years), with RCM in New York Heart Association Functional Classification III and IV (unknown cause in 3 and a positive family history in 1 patient) with pulmonary hypertension (mean pulmonary artery pressure [PAP] 34 10 mm Hg, mean PCWP, 22 7 mm Hg, mean right atrial pressure [RAP] 6 3 mm Hg), and four patients, aged 2.9 years (range, 2.3–4.0 years), with DCM in New York Heart Association III and IV (2 patients each with idiopathic DCM and chronic lymphocytic myocarditis), who presented with severely impaired LV function (mean LV ejection fraction, 21% 7 %; mean PAP, 17 3 mm Hg; mean PCWP, 13 3 mm Hg; and mean RAP, 5 3 mm Hg). In all cases, a percutaneous atrial septostomy using Brockenbrough technique was created without adverse events. Immediately after the procedure, PCWP dropped to 14 3 mm Hg in the RCM group and to 8 2 mm Hg in the DCM group, whereas RAP remained stable (6 2 mmHg).

Giant remnant of fetal circulation leading to cyanosis: pseudo-cor triatriatum dexter

Remnants of the right valve of the sinus venosus, which fail to regress may present as an obstructing membrane within the right atrium as a very rare congenital anomaly. When associated with an atrial septal defect, primary cyanosis may occur due to a large right-to-left shunt. We report on a 1-week old girl with a pseudo-cor triatriatum dexter and immediate cyanosis after an uncomplicated birth. She was successfully treated with surgical excision of the obstructing membrane.

Perinatal outcomes of congenital heart disease after emergent neonatal cardiac procedures

Abstract Purpose: We compared outcome of neonates with prenatal and post-natal diagnosis of congenital heart disease presenting in our paediatric heart centre between March 2005 and May 2015 who underwent an emergent intervention within 48 h post-partum. Materials and methods: In 52/111 (46.8%) with emergent intervention, congenital heart disease was diagnosed prenatally, in 59/111 (53.2%) with no specialized foetal echocardiography, diagnosis was made post-natally. In 98/111 (88.2%), 30-day outcome was known. Results: Regarding the entire cohort, 30-day survival did not differ significantly in prenatal and post-natal diagnosis group (71.2 vs. 72.9%; p > .1). Infants with prenatal diagnosis were more likely to be born by caesarean section (59.6% vs. 33.9%, p = .01). Those with post-natal diagnosis had a higher need for intubation (32.7% vs. 52.5%; p < .05). Subgroup analysis of HLH/HLHC (hypoplastic left heart/hypoplastic left heart complex) patients revealed higher number of deaths within 30 days of life in the post-natal diagnosis group, although the difference did not reach statistical significance (5/7, 71.4% vs. 5/20, 25.0%; p = .075). Conclusion: For newborns who require emergent neonatal cardiac procedures, our results point towards a lower death rate after prenatal diagnosis of HLH/HLHC.