Multiprofessional Critical Care Review: Pediatric 2024 (MCCRCP24ON)-Congenital Cardiac Malformations

Video Transcription

Video Summary

Dr. Ravi Theogarajan, a cardiac intensivist from Boston Children's, discusses key aspects of congenital heart disease (CHD) relevant for exams and clinical practice. CHD is one of the most common congenital malformations in children, requiring ICU care and interventions in about one-third of cases. Many patients with CHD have associated genetic syndromes like DeGeorge syndrome, characterized by hypocalcemia, immune deficiency, and conotruncal abnormalities (e.g., tetralogy of Fallot).<br /><br />Diagnosis is primarily through echocardiography, supplemented by CTs and MRIs for surgical planning. Cardiac catheterization is mainly used for interventional purposes. Key formulas include calculating cardiac output using the Fick principle and estimating left-to-right shunts via the QP/QS ratio.<br /><br />Theogarajan reviews two common CHDs—tetralogy of Fallot and single ventricle defects. Tetralogy of Fallot involves a VSD, right ventricular outflow obstruction, aortic override, and right ventricular hypertrophy, leading to mixed blood flow and potential cyanotic "TET spells." Treatment includes surgical repair within six months.<br /><br />Single ventricle defects, particularly hypoplastic left heart syndrome (HLHS), require balancing pulmonary and systemic blood flows. HLHS involves a single ventricle managing mixed systemic and pulmonary circulation. Management includes staged surgeries (Norwood, Glenn, and Fontan procedures) to transition from parallel to series circulations and balancing systemic and pulmonary vascular resistances post-operation.

Keywords

congenital heart disease

tetralogy of Fallot

single ventricle defects

hypoplastic left heart syndrome

cardiac catheterization

echocardiography

DeGeorge syndrome

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