Key result
Surgical pericardial patch repair of IVC sinus venosus ASD reduces pulmonary pressures by ~18 mmHg.
Why the study?
Sinus venosus ASD is a rare congenital defect accounting for 5% to 10% of all ASDs that requires immediate identification and treatment to prevent significant left atrial dilatation and dyspnea.
Case Report (n=1)
No
Early echocardiographic detection and surgical pericardial patch repair of rare inferior vena cava-type sinus venosus ASD in an adolescent presenting with chronic respiratory infections effectively reduced right ventricular overload and lowered systolic pulmonary artery pressures to 25-30 mm Hg.
Answers are grounded in this paper and cite their sources.
Supports surgical repair of rare IVC-type sinus venosus ASD in adolescents with PH; leaves open need for prospective validation.
RATIONALE: Overall, 5% to 10% of all atrial septal defects (ASDs) are of the sinus venosus type, a rare congenital disease. This defect must be identified and treated immediately since it might cause significant left atrial dilatation and dyspnea. PATIENT CONCERNS: A 15-year-old male presented with previously undetected sinus venosus type ASD. The patient arrived with a high-grade heart murmur and a history of recurring respiratory illnesses. DIAGNOSES: A clinical examination identified a 3/6 systolic ejection murmur in the second left intercostal gap and a fixed split second heart sound. Electrocardiography revealed sinus rhythm with partial right bundle branch block, while echocardiography indicated considerable enlargement of the right atrium and right ventricle, accompanied by a D-shaped left ventricle. A sinus venosus type ASD with an inferior vena cava-right atrial shunt was verified. INTERVENTIONS: The patient had surgical closure of the defect via midline sternotomy, thereafter gaining access to the right atrium and performing pericardial patch repair. OUTCOMES: Two months postoperatively, the patient's right ventricular diameters and pulmonary artery pressures were significantly diminished (25-30 mm Hg), and his overall status was excellent. LESSONS: Sinus venosus type ASD is an infrequent etiology of persistent respiratory problems in adolescents. Early detection by thorough clinical and echocardiographic assessment, followed by timely surgical intervention, may lead to superior functional recovery and improved long-term quality of life.
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Alhamid et al. (2025) conducted a case report in Sinus venosus type atrial septal defect (ASD) with inferior vena cava-right atrial shunt (n=1). Surgical pericardial patch repair via midline sternotomy was evaluated on Systolic pulmonary artery pressure and right ventricular dimensions at 2-month follow-up. Surgical pericardial patch repair of an inferior vena cava sinus venosus ASD reduced systolic pulmonary artery pressure from ~45 mm Hg to 25-30 mm Hg at 2 months in a 15-year-old male.
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