Description
Echocardiographic evaluation plays a vital role in diagnosing and managing congenital heart disease (CHD). It provides real-time, non-invasive imaging to assess cardiac structure, function, and blood flow. Key modalities include two-dimensional (2D), Doppler, and color flow imaging, which help visualize septal defects, valve anomalies, and outflow tract obstructions. Fetal echocardiography allows prenatal detection, while transthoracic echocardiography (TTE) is commonly used in neonates and children. Transesophageal echocardiography (TEE) is valuable in complex cases and intraoperative settings. Accurate echocardiographic assessment guides clinical decisions, surgical planning, and long-term follow-up, making it an essential tool in the comprehensive care of patients with CHD.
Summary Listen
- A pediatrician with a focus on non-invasive pediatric cardiology and echocardiography presents a case study of a two-month-old female infant diagnosed with a heart condition. The infant, born at 39 weeks via C-section, presented with failure to thrive, distress, and a grade 3 systolic murmur. Initial investigations revealed mild metabolic acidosis, increased pulmonary vascular markings on chest X-ray, and wide axis deviation with biventricular hypertrophy on ECG.
- Echocardiography revealed a ventricular septal defect (VSD) and that both the aorta and pulmonary artery originated predominantly from the right ventricle. A large sub-aortic VSD was identified with significant overriding of the aorta. The patient also had a patent ductus arteriosus (PDA) and severe pulmonary artery hypertension.
- The final diagnosis was double outlet right ventricle (DORV), specifically a sub-aortic variant. The presentation and diagnostic tools were discussed, including echocardiographic evaluation, but management was only touched on briefly. DORV is a rare congenital heart defect where both great arteries arise from the right ventricle.
- Embryologically, DORV results from a defect in conotruncal septation during cardiac development. It accounts for a small percentage of congenital heart defects and is often diagnosed during infancy due to cyanosis, heart failure, or failure to thrive. Prenatal detection is increasing. 50% of DORV cases have other cardiac defects and are linked to genetic syndromes like Down syndrome.
- Echocardiography is the primary diagnostic tool. The key finding is both arteries arising from the right ventricle, either completely or with significant overriding. Distinguishing DORV from tetralogy of Fallot involves considering the degree of overriding and the presence of aortomitral continuity. Associated pulmonary stenosis and VSD type are important to identify.
- Different types of echocardiography were explained. Standard views in pediatric echocardiography include subcoastal, parasternal, apical, and suprasternal views. Hemodynamic assessment is performed using color flow Doppler and spectral Doppler. Chamber quantification is done using software to measure heart size and ejection fraction.
- Management depends on the anatomy, physiology, and presentation. Initial stabilization with ABCDE management is essential in unstable children. Medications like diuretics, ACE inhibitors, beta-blockers, and inotropes are used. Surgical repair is the ultimate goal, aiming for the best possible outcome and long-term survival. Procedures include VSD closure, arterial switch operations, and the Fontan procedure for single-ventricle physiology. Lifelong cardiology follow-up is necessary.
Sample Certificate
About the Speakers
Dr. Dhruba Shrestha
Senior Consultant Pediatrician and Head of Department at Siddhi Memorial Hospital,Nepal
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