Author + information
- Guiomar Mendieta, MD, MSc∗ (, )
- Silvia Montserrat, MD, PhD,
- Susanna Prat-Gonzalez, MD, PhD,
- Bàrbara Vidal, MD, PhD and
- Marta Sitges, MD, PhD
- Cardiology Department, Cardiovascular Institute, Hospital Clínic, Institut d'Investigaciones Biomèdiques August Pi i Sunyer, University of Barcelona, Spain
- ↵∗Address for correspondence:
Dr. Guiomar Mendieta, Cardiology Department, Cardiovascular Institute, Hospital Clínic de Barcelona, c/Villarroel 170, 08036 Barcelona, Spain.
A 35-year-old female athlete presented with recent episodes of pre-syncope on exertion while exercising. An isolated double-chambered right ventricle was diagnosed by transthoracic echocardiography and cardiac magnetic resonance. This defect typically presents during infancy and/or early childhood, is rarely reported in adults, and is usually associated with other congenital defects. (Level of Difficulty: Advanced.)
A 35-year-old female athlete with no medical history other than a childhood murmur, presented with a few recent episodes of pre-syncope on exertion while exercising. The physical examination revealed a palpable thrill with a right parasternal heave and a loud systolic grade-5 murmur along the lower left parasternal border on auscultation. Electrocardiography showed right-axis deviation and an incomplete right bundle branch block, with prominent R waves in right precordial leads. Transthoracic echocardiography showed a mildly enlarged right ventricle (RV) with mid-ventricular hypertrophy and a membranous band in the outflow tract (Figure 1, arrow) generating an obstructive gradient that was unmeasurable due to difficulty in Doppler wave alignment. Tricuspid regurgitation estimated severe right intraventricular obstruction (65 mm Hg mean gradient). Cardiac magnetic resonance confirmed the presence of a subinfundibular band extending from the interventricular septum to the right ventricle free wall, dividing the chamber into 2 cavities, as shown by the cardiac magnetic resonance sagittal (upper strip) and short axis (lower strip) planes in Figure 2. Mild dilation of the infundibulum (3 cm) and mild pulmonary regurgitation were observed. Late gadolinium enhancement identified fibrosis within the septal muscular band (Figure 3). No ventricular septal defect was identified.
A double-chambered RV was diagnosed, an infrequent congenital entity characterized by an anomalous fibrous band that divides the RV into a high-pressure and proximal chamber and another low-pressure distal cavity (1). Some authorities, however, consider it an acquired congenital cardiac defect, given that a genetic predisposition to abnormal band formation is suspected, although evidence for the latter has not been clearly shown (2). This defect typically presents during infancy and/or early childhood and is rarely reported in adults. Other associated congenital defects, such as perimembranous ventricular septal defects, are frequently encountered. Adult patients may develop progressive dyspnea on exertion due to increasing RV outflow tract obstruction. If mid-RV obstruction is significant, surgical intervention is required. Long-term outcomes however are unclear (3).
The authors have reported that they have no relationships relevant to the contents of this paper to disclose.
- Abbreviations and Acronyms
- right ventricle
- ventricular septal defect
- Received April 25, 2019.
- Accepted May 23, 2019.
- 2019 The Authors