Mortality outcomes associated with surgical palliation for hypoplastic left heart syndrome (HLHS) have continued to improve with time. Despite these advances, interstage mortality continues to be difficult to predict and prevent. This case report details a 3-year-old boy with HLHS palliated with a Glenn shunt who presented with acute myocardial infarction due to near total occlusion of the distal right coronary artery. He was treated by thrombus aspiration with good clinical response. This is the first report of thrombus aspiration in complex pediatric congenital heart disease and demonstrates the utility of collaboration between congenital and adult cardiologists.
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http://dx.doi.org/10.1002/ccd.25362 | DOI Listing |
Multimed Man Cardiothorac Surg
January 2025
Congenital Heart Center, Division of Cardiovascular Surgery, Department of Surgery, University of Florida, Gainesville, FL, USA.
The Berlin Heart EXCOR is a pulsatile paracorporeal ventricular assist device (VAD) for neonates, infants, children and adults with congenital or acquired severe ventricular dysfunction. Berlin Heart EXCOR VADs are routinely used as either a bridge to a cardiac transplantation, or occasionally as a bridge to ventricular recovery. Our programmatic philosophy is to bridge neonates and infants with functionally univentricular ductal-dependent systemic circulation or functionally univentricular ductal-dependent pulmonary circulation who are at high risk for staged palliation because of important cardiac risk factors with a single-ventricle VAD (sVAD) as a bridge to a cardiac transplant.
View Article and Find Full Text PDFEur J Cardiothorac Surg
January 2025
Division of Cardiovascular Surgery, The Labatt Family Heart Centre, The Hospital for Sick Children, Toronto, ON, Canada.
Objectives: This study aimed to assess the outcomes of heterotaxy patients undergone the Fontan operation, focusing on morphological features and surgical techniques.
Methods: Eighty-two consecutive heterotaxy patients who underwent the Fontan operation from 1985 to 2021 were compared to 150 patients with tricuspid atresia (TA) and 144 patients with hypoplastic left heart syndrome (HLHS). The Kaplan-Meier method and Cox proportional hazard model were used to analyze transplant-free survival and predictor of outcomes.
Eur Heart J Case Rep
January 2025
Department of Pediatric Cardiology, Osaka Women's and Children's Hospital, Izumi, Osaka 594-1101, Japan.
Background: A partial atrioventricular septal defect (AVSD) with a hypoplastic left ventricle and common atrium is a rare combination of cardiac anomalies that can be associated with Ellis-van Creveld (EVC) syndrome.
Case Summary: A female neonate with EVC syndrome was diagnosed with an unbalanced AVSD and hypoplastic left ventricle. Pulmonary artery banding and ductus ligation were performed at 23 days after birth.
Cardiol Young
January 2025
Department of Pediatric Cardiovascular Surgery, Osaka City General Hospital, Osaka, Japan.
A patient with borderline left ventricle successfully underwent biventricular repair following a staged surgical approach to promote left ventricular growth. Despite initial concerns about left ventricle size, apex formation and adequate size of atrioventricular valve indicated potential for future growth. The patient demonstrated significant left ventricular growth, resulting in stable biventricular circulation and a favourable outcome over a three-year postoperative follow-up period.
View Article and Find Full Text PDFAm J Med Genet A
January 2025
Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy.
TBCK (TBC1 Domain-Containing Kinase) encodes a protein playing a role in actin organization and cell growth/proliferation via the mTOR signaling pathway. Deleterious biallelic TBCK variants cause Hypotonia, infantile, with psychomotor retardation and characteristic facies 3. We report on three affected sibs, also displaying cardiac malformations.
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