A case of malignant catatonia with idiopathic pulmonary arterial hypertension treated by electroconvulsive therapy.

BACKGROUND: Idiopathic pulmonary arterial hypertension (IPAH) is a progressive and fatal cardiovascular disease if left untreated. In patients with IPAH with psychiatric illness or other complications, careful attention is required when administering medical therapies that may affect their hemodynamics. Patients suffering from IPAH who undergo anesthesia and surgery have a high mortality and morbidity rate. We describe the treatment of intractable psychiatric symptoms with electroconvulsive therapy (ECT) in a patient with IPAH. CASE PRESENTATION: A 23-year-old woman with IPAH and type I diabetes mellitus (DM) presented with malignant catatonia. Her heart function was classified as New York Heart Association (NYHA) class III. She required a rapid cure and ECT due to various psychiatric symptoms resistant to conventional medications. Pulmonary hypertensive (PH) crisis is the most concerning complication that can be induced by the sympathetic stimulation of ECT. To avoid PH crisis, we administered oxygen using a laryngeal mask and administered remifentanil for anesthesia. We also prepared standby nitric oxide for possible PH crisis, although it was ultimately not needed. With 14 ECT sessions, her malignant catatonia was ameliorated without physical complications. CONCLUSION: ECT is an acceptable option for the treatment of medication-refractory psychiatric disturbances in patients with IPAH, provided careful management is assured to prevent or address complications.

Effectiveness of carvedilol for congestive heart failure that developed long after modified Fontan operation.

We report a case of a patient with severe heart failure after Fontan procedure in whom carvedilol was very effective. A 27-year-old man had intractable congestive heart failure due to severe ventricular dysfunction after Fontan operation. Central venous pressure was elevated to 29 mmHg. A right-to-left shunt was noted across a large collateral vessel between the innominate vein and the pulmonary vein. He was administered carvedilol (initial dose, 2 mg/day; maximum dose, 30 mg/day). Cardiac catheterization performed 1 year after carvedilol administration revealed a decrease in atrial pressure and improvement of ventricular function. He underwent a conversion operation to total cavopulmonary connection (TCPC) and ligation of a collateral vein communicating with the innominate and pulmonary veins. Carvedilol may be a legitimate treatment before TCPC conversion or heart transplantation for the high-risk group of patients with a failed Fontan circulation.

Pathological lesions causing pulmonary hypertension after closure of a ventricular septal defect.

A 15-year-old boy with a ventricular septal defect, pulmonary hypertension, Down's syndrome, and extremely thickened media (ETM) of the small pulmonary arteries died of heart failure and pulmonary hypertension 13 years after intracardiac repair. Microscopic examination of lung specimens collected prior to the intracardiac repair and at the time of autopsy revealed that the ETM had remained unchanged and that the arteries connected to the vessels with ETM had become severely thickened. The present case shows that even a small percentage of arteries with ETM can cause pulmonary hypertension, and illustrates one of the mechanisms of how pulmonary hypertension can fail to be resolved after intracardiac repair.