Myocardial Calcification and the Demise of an Infant With Surgically Treated Hypoplastic Left Heart Syndrome.
Pediatr Dev Pathol
; 24(1): 68-72, 2021.
Article
em En
| MEDLINE
| ID: mdl-33433253
ABSTRACT
A term female infant with hypoplastic left heart syndrome underwent Norwood palliation including aortic and pulmonary amalgamation with arch reconstruction, atrial septectomy, and right ventricle to pulmonary artery conduit. Postoperatively, she experienced hypoxemia and lactic acidosis although echocardiogram showed adequate conduit function. She was placed on veno-arterial extracorporeal membrane oxygenation (ECMO) on postoperative day two with improvement. ECMO decannulation was attempted with subsequent cardiac arrest and ultimate failure to resuscitate, eleven days after surgery. Autopsy confirmed clinical findings and evidence of surgical intervention with a patent conduit and neo-aorta. Multiple subendocardial right ventricular dystrophic calcifications involving the outflow tract were identified grossly and histologically with foci of associated myonecrosis. Myocardial calcification may lead to abnormal heart wall motion by increasing rigidity and compromising myocyte function or compromising the conduction system. In this patient, right ventricular turbulence caused by systolic and diastolic flow patterns, including mild tricuspid regurgitation, may have played a role in inducing dystrophic calcification along with surgery and ECMO dependence. Compromised myocyte function from calcifications, right ventricular hypertrophy, lung immaturity, and persistent pulmonary hypertension were likely sources of cardiac strain leading to the patient's demise. This case represents a previously unreported complication of hypoplastic left heart syndrome treatment.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Calcinose
/
Síndrome do Coração Esquerdo Hipoplásico
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Procedimentos de Norwood
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Miocárdio
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
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Prognostic_studies
Limite:
Female
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Humans
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Newborn
Idioma:
En
Revista:
Pediatr Dev Pathol
Assunto da revista:
PATOLOGIA
/
PEDIATRIA
Ano de publicação:
2021
Tipo de documento:
Article