Abstract 18136: The Tale of Two Chambers : Late Diagnosis of a Young Condition
A 41-year-old female with a small, asymptomatic ventricular septal defect (VSD), obesity, and asthma presented with 2 months of dyspnea on exertion and palpitations. Her symptoms were initially thought to be an asthma exacerbation. However, due to progression of symptoms, a transthoracic echocardiogram (TTE) was performed, which confirmed a VSD. A 24-hour holter monitor found 11 beats of wide complex tachycardia. She was subsequently referred to our hospital for evaluation of VSD closure.
On admission, her vital signs were normal, physical exam revealed a 3/6 holosystolic murmur across the precordium. An electrocardiogram exhibited right axis deviation and right ventricular (RV) hypertrophy. TTE noted a hypokinetic, dilated RV; signs of RV pressure overload, restrictive supra-cristal VSD and normal left ventricle. RV systolic pressure could not be estimated. The RV failure was not explained by a VSD (without Eisenmenger syndrome). Hence, the differential included pulmonary hypertension, RV outflow tract (RVOT) obstruction or pulmonary valve (PV) stenosis. Right and left heart catheterization displayed an RV pressure of 161/15 mmHg, RV to pulmonary artery (PA) gradient of 115 mm Hg, Qp/Qs of 0.92 and no step up of oxygen. Pulmonary vascular resistance, LV end diastolic pressure and coronary arteries were unremarkable. The differential narrowed down to PV stenosis, Tetralogy of Fallot (typically membranous and large VSDs), double chamber right ventricle (DCRV) or supravalvular stenosis (PA membrane / stenosis). Cardiac magnetic resonance imaging revealed thick muscle bands dividing the RV into two chambers, causing severe stenosis. Diagnosis of DCRV was established. Given the symptoms and severity of stenosis, she underwent a successful surgical muscle band resection with resolution of symptoms and RV dysfunction.
We present an unusual case of adult presentation of DCRV. Our case highlights the progressive nature of DCRV and the (often) delayed diagnosis in adults due to non-specific symptoms and comorbidities. The RVOT is not routinely examined / inadequately visualized on TTE making the diagnosis challenging. Hemodynamic assessment and multimodality imaging aid diagnosis. Surgical repair is indicated for symptoms and peak gradients > 60 mmHg.
Author Disclosures: A. Dwivedi: None. C. Weinberg: None. A. Jung: None.
- © 2016 by American Heart Association, Inc.