Acyanotic Congenital Heart Disease, repaired, evolves into a Cyanotic Congenital Heart Disease and presents with an atrial tachycardia

Ramya Rajesh

MEM Resident, Kauvery Hospitals, Trichy


Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. [1] The previous left-to-right shunt is converted into a right-to-left shunt secondary to elevated pulmonary artery pressures and associated pulmonary vascular disease.

Lesions in Eisenmenger syndrome, such as large septal defects, are characterized by high pulmonary pressure and/or a high pulmonary flow state. Development of the syndrome represents a point at which pulmonary hypertension is irreversible and is an indication that the cardiac lesion is likely inoperable Cardiac arrhythmias and sudden cardiac death are important late complications of this syndrome. Conservative management with medications and/or lung and cardiac transplantation are therapeutic approaches that can offer quality-of-life improvement.

We report a patient who has undergone a device closure of a VSD several years earlier, which gradually failed, leading to severe pulmonary artery hypertension and a cyanotic state

Case Presentation

The patient was a 24-years-aged, euglycemic, normotensive woman who was known to have had an acyanotic congenital heart disease ventricular septal defect patient. She had undergone surgical closure of a ventricular septal defect in 2006 and was asymptomatic until she presented to our emergency department with H/O complaints of palpitation for one day.

On examination, she was conscious and oriented. Her heart rate was 190 per minute, irregular and low volume, blood pressure was 90/60 mmhg and saturation was 90% on room air. She was tachypnoeic with a respiratory rate of 30/min. Cyanosis was noted in her palms, lips and tongue.

Electrocardiogram showed Atrial flutter changes.



Fig. 1. Atrial flutter with varying conduction block, RBBB pattern and RVH.

Echocardiography showed severe pulmonary artery hypertension. Hence the patient was admitted to the intensive care unit and started on intravenous cordarone, ionotropes, pulmonary vasodilators, diuretics, and with oxygen support.


The patient was medically stabilised, weaned off from oxygen support, planned for electrophysiological study and radiofrequency ablation in the next admission and discharged in hemodynamically stable condition.


Later patient was readmitted after one week. Electrophysiological study was conducted which showed right atrial Cristal atrial tachycardia (right atrial tachycardia from the crista terminalis). Focal ablation using 7.5 F flexibility irrigation catheter and focal Cristal atrial tachycardia was ablated post-procedure hospital stay was uneventful and the patient got discharged in stable condition.


Penalver, J L., et al. Eisenmenger Syndrome.