ABSTRACT
A 38-year-old man with a functional single ventricle secondary to hypoplastic left heart syndrome presented with exertional fatigue. His last palliation was an intra-atrial conduit Fontan procedure. Comprehensive evaluation showed elevated liver enzyme values and a small, calcified conduit. Successful conversion to a nonfenestrated extracardiac conduit Fontan was performed with normalization of his liver enzyme values.
ABSTRACT
Cardiac involvement as a complication of severe acute respiratory syndrome coronavirus 2 infection in children is a relatively new entity. We present our initial experience managing children with coronavirus disease 2019-related acute myocardial injury. The 3 patients presented here represent a spectrum of the cardiac involvement noted in children with coronavirus disease 2019-related multisystem inflammatory syndrome, including myocarditis presenting as cardiogenic shock or heart failure with biventricular dysfunction, valvulitis, coronary artery changes, and pericardial effusion.
Subject(s)
Betacoronavirus , Coronavirus Infections , Heart Failure , Heart Valve Diseases , Myocarditis , Pandemics , Patient Care Management/methods , Pericardial Effusion , Pneumonia, Viral , Systemic Inflammatory Response Syndrome , Adolescent , Betacoronavirus/isolation & purification , Betacoronavirus/pathogenicity , COVID-19 , Cardiac Imaging Techniques/methods , Child , Child, Preschool , Coronary Vessels/diagnostic imaging , Coronary Vessels/pathology , Coronavirus Infections/diagnosis , Coronavirus Infections/physiopathology , Coronavirus Infections/therapy , Heart Failure/etiology , Heart Failure/therapy , Heart Valve Diseases/diagnosis , Heart Valve Diseases/virology , Humans , Myocarditis/therapy , Myocarditis/virology , Pericardial Effusion/therapy , Pericardial Effusion/virology , Pneumonia, Viral/diagnosis , Pneumonia, Viral/physiopathology , Pneumonia, Viral/therapy , SARS-CoV-2 , Shock, Cardiogenic/etiology , Shock, Cardiogenic/therapy , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/physiopathology , Systemic Inflammatory Response Syndrome/therapy , Treatment OutcomeABSTRACT
During the past decade, a hybrid procedure has emerged and dramatically evolved as an alternative stage I palliation to the conventional Norwood procedure in neonates with hypoplastic left heart syndrome (HLHS). The hybrid approach avoids the need for cardiopulmonary bypass (CPB) utilizing stenting of the arterial duct and bilateral pulmonary artery banding. Cerebral and coronary perfusion pressure is maintained, and the pulmonary vasculature is protected from higher systemic pressure. Elimination of risks associated with CPB gains vital time to stabilize the patient and correct coexisting noncardiac anomalies and allows growth in preparation for the later stages of the Fontan pathway. The association of HLHS with right congenital diaphragmatic hernia (CDH) is rare. We report performing a successful hybrid stage I palliation on a neonate with HLHS and severe right CDH.
Subject(s)
Hernias, Diaphragmatic, Congenital/surgery , Hypoplastic Left Heart Syndrome/surgery , Stents , Cardiopulmonary Bypass/adverse effects , Cardiopulmonary Bypass/methods , Female , Hernias, Diaphragmatic, Congenital/physiopathology , Humans , Hypoplastic Left Heart Syndrome/physiopathology , Infant, Newborn , Severity of Illness Index , Treatment OutcomeABSTRACT
Myocardial ischemia due to coronary artery disease is an extremely rare condition in childhood and adolescence. Absence of obvious serious risk factors remains a challenge to modern cardiology. We present the case of a 14-year-old boy who underwent quadruple-vessel coronary artery bypass grafting with bilateral pedicled internal mammary artery and bilateral radial artery grafting. We try to highlight a rare but important 4G variant PAI-1 (SERPINE 1) gene mutation as the etiology of severe coronary artery disease in our patient. To the best of our knowledge, he is one of the youngest patients who underwent coronary artery bypass surgery with 4 arterial grafts.
Subject(s)
Coronary Artery Bypass/methods , Coronary Artery Disease/surgery , Plasminogen Activator Inhibitor 1/genetics , Polymorphism, Genetic , Adolescent , Coronary Artery Disease/genetics , Electrocardiography , Humans , MaleABSTRACT
Endothelial caveolin-1 loss is an important feature of pulmonary hypertension (PH); the rescue of caveolin-1 abrogates experimental PH. Recent studies in human PH suggest that the endothelial caveolin-1 loss is followed by an enhanced expression of caveolin-1 in smooth muscle cells (SMC) with subsequent neointima formation. In order to evaluate caveolin-1 expression in infants with PH, we examined the available clinical histories, hemodynamic data, and the expression of caveolin-1, PECAM-1, vWF, and smooth muscle α-actin in the lung biopsy/autopsy specimens obtained from infants with congenital heart disease (CHD, n = 8) and lung disease (n = 9). In CHD group, PH associated with increased pulmonary blood flow exhibited loss of endothelial caveolin-1 and PECAM-1 in pulmonary arteries; additional vWF loss was associated with enhanced expression of caveolin-1 in SMC. In the absence of PH, increased or decreased pulmonary blood flow did not disrupt endothelial caveolin-1, PECAM-1, or vWF; nor was there any enhanced expression of caveolin-1 in SMC. In Lung Disease + PH group, caveolin-1, PECAM-1, and vWF were well preserved in seven infants, and importantly, SMC in these arteries did not exhibit enhanced caveolin-1 expression. Two infants with associated inflammatory disease exhibited loss of endothelial caveolin-1 and PECAM-1; additional loss of vWF was accompanied by enhanced expression of caveolin-1 in SMC. Thus, associated flow-induced shear stress or inflammation, but not elevated pulmonary artery pressure alone, disrupts endothelial caveolin-1. Subsequent vWF loss, indicative of extensive endothelial damage is associated with enhanced expression of caveolin-1 in SMC, which may worsen the disease.