Abstract
A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air.
Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular
dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal.
Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures,
and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to
reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right
pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and
pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful;
oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no
ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting
defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular
exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched
foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one
of the options in such patients.
Keywords
arrhythmia, atrial septal defect (ASD), cardiomyopathy, cavopulmonary anastomosis, sudden cardiac death, magnetic resonance
imaging (MRI), off pump surgery, ventricle, right, cyanosis
venous pressure (CVP) was 17 mm Hg. There was evidence of the literature.8 Genetic analysis, one of the diagnostic criteria,
fibrosis over the RV with good sized main and branch PAs. She was not carried out in the case we present here.1 The manage-
underwent off-pump cavopulmonary anastomosis between ment of ARVD still remains challenging and requires a flexible
right superior vena cava and right PA. Her postoperative period approach, according to the presentation of the patient. Modified
was uneventful and her saturation improved to 89%. She Glenn shunt can be a good palliation in patients with severe
remained in the intensive care unit for 2 days and was dis- cyanosis and reduced pulmonary blood flow. Since closure of
charged home 1 week after the surgery. Patient is asympto- the ASD and completion of one-and-a-half ventricle repair
matic at 18-month follow-up. Since there were no ventricular would have required extracorporeal circulation with its atten-
arrhythmia on follow-up, implantation of cardioverter defibril- dant effects on myocardial and lung function, we did not opt for
lator was deferred. it.