Not all obstructive cardiac lesions are created equal

Double-chamber right ventricle in pregnancy

Sandhya Murthy, George Lui, Farbod Raiszadeh, Lawrence Boxt, Cynthia C. Taub

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy. Double-chambered right ventricle (DCRV) is a rare form of right ventricular outfl ow tract (RVOT) obstruction consisting of a muscle bundle that divides the right ventricle into a sinus (inlet) and infundibulum (outlet). The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. Most cases are diagnosed in early childhood, and few cases are discovered in adulthood. This poses a unique diagnostic challenge for physicians, as it is commonly mistaken for other common acquired disease states. We describe the course of a young adult with DCRV during pregnancy.

Original languageEnglish (US)
JournalEchocardiography
Volume29
Issue number8
DOIs
StatePublished - Sep 2012

Fingerprint

Heart Ventricles
Pregnancy
Ventricular Outflow Obstruction
Muscles
Pituitary Gland
Young Adult
Hemodynamics
Pressure
Ventricular Remodeling
Ventricular Pressure
Diagnostic Errors
Pulmonary Hypertension
Echocardiography
Heart Diseases
Blood Pressure
Physicians

Keywords

  • congenital heart disease
  • double-chamber right ventricle
  • pregnancy
  • pulmonary hypertension

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Radiology Nuclear Medicine and imaging

Cite this

Not all obstructive cardiac lesions are created equal : Double-chamber right ventricle in pregnancy. / Murthy, Sandhya; Lui, George; Raiszadeh, Farbod; Boxt, Lawrence; Taub, Cynthia C.

In: Echocardiography, Vol. 29, No. 8, 09.2012.

Research output: Contribution to journalArticle

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