Key result
Extreme biventricular hypertrophy in infant D-TGA leads to fatal collapse prior to surgical septectomy.
Why the study?
Co-occurrence of D-TGA and severe idiopathic biventricular hypertrophic cardiomyopathy in late infancy creates unique physiological barriers and surgical limitations not previously well characterized.
Case Report (n=1)
No
This sentinel case highlights that late-presenting non-diabetic biventricular hypertrophy combined with D-TGA renders traditional surgical therapies physiologically intolerable, suggesting formal surgical atrial septectomy as a potential, though highly precarious, salvage pathway.
Alerts clinicians to extreme fragility before atrial septectomy in late D-TGA with non-diabetic hypertrophy; leaves open optimal salvage strategies.
Background: Co-occurrence of dextro-transposition of the great arteries (D-TGA) and severe hypertrophic cardiomyopathy (HCM) is an exceptionally rare and hemodynamically precarious scenario. While transient, reversible HCM is well-documented in neonates of diabetic mothers, idiopathic biventricular HCM presenting late in infancy introduces unique physiological barriers. Case Presentation: We present the case of a 4-month-old, non-diabetic infant diagnosed with parallel circulation D-TGA, complicated by massive biventricular hypertrophy, a large ventricular septal defect, and flow-related pulmonary hypertension. Discussion: The contemporaneous presentation of these pathologies creates a vicious hemodynamic cycle: the excessive volume and pressure loading from unrestrictive shunts collide with severe intrinsic biventricular hypertrophy, culminating in profound diastolic dysfunction and severely restricted inter-circulatory mixing. This case underscores that, unlike transient neonatal HCM, late-presenting non-diabetic hypertrophy combined with high-volume pulmonary overcirculation renders traditional bridging and corrective therapies physiologically intolerable. Conclusion: We detail the profound surgical limitations in this subset of patients - specifically the theoretical contraindications for pulmonary artery banding, the Arterial Switch Operation, and atrial switch procedures. We highlight why formal surgical atrial septectomy, with or without patent ductus arteriosus ligation, is proposed as a potential salvage pathway to navigate such profound physiological fragility when advanced percutaneous interventions are unavailable.
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Kanaan et al. (2026) conducted a case report in Dextro-transposition of the great arteries and severe hypertrophic cardiomyopathy (n=1). Urgent surgical atrial septectomy (planned) was evaluated. A 4-month-old non-diabetic infant with D-TGA and extreme biventricular hypertrophy suffered fatal hemodynamic collapse before a planned urgent surgical atrial septectomy could be performed.
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