Pulmonary arterial hypertension with frequent syncope: Clinical case presentation

Basha, Aseem; Krishnan, Shanmugam; Gujral, Jaskaran Singh; Goswami, Karan

doi:10.4103/jpcs.jpcs_39_18

Cited by 1 publication

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“… 6 Severe RV failure causes syncope due to (a) decreased RV-Left ventricular interdependence, (b) RV-Pulmonary artery uncoupling and (c) resultant abrupt drop in systemic pressures due to decrease in stroke volume in the setting of fixed downstream obstruction and pulmonary hypertension. 7 , 8 Arrhythmias can occur due to RV failure and right atrial dilation 9 or recurrent pulmonary emboli. 10 Syncope is a reported presenting symptom in PE 11 as well as PTTM.…”

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confidence: 99%

Pulmonary tumor thrombotic microangiopathy presenting as recurrent syncope

Calderoni

Moretta

Merrill-Henry

et al. 2020

SAGE Open Medical Case Reports

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Pulmonary tumor thrombotic microangiopathy is a rare condition in which embolization of tumor cells to the pulmonary arterioles causes fibrocellular intimal thickening and activation of the coagulation cascade resulting in pulmonary hypertension and right heart failure. Herein, we highlight a young 35-year-old male with no known past medical history who presented with recurrent syncope and dyspnea, and was found to have severe right heart failure and pulmonary hypertension. He developed sudden clinical deterioration and died after a cardiac arrest. Autopsy revealed poorly differentiated gastric adenocarcinoma and pulmonary tumor thrombotic microangiopathy. New onset severe pulmonary hypertension and right heart failure without any other obvious etiology should encourage the reader to evaluate for pulmonary tumor thrombotic microangiopathy and undergo a diligent search for underlying malignancy. This case highlights recurrent syncope as a rare presentation of this rapidly fatal disease.

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