Pulmonary hypertension secondary to pulmonary veno-occlusive disease complicated by right heart failure, hypotension and acute kidney injury

Abstract

Pulmonary veno-occlusive disease (PVOD) is rare condition which can lead to severe pulmonary hypertension, right ventricular dysfunction, and cardiopulmonary failure. The diagnosis of PVOD can be challenging due to its nonspecific symptoms and its similarity to idiopathic pulmonary arterial hypertension and interstitial lung disease in terms of diagnostic findings. This case describes a 57 year old female patient who presented with a 5-month history of progressive dyspnea on exertion and nonproductive cough. Workup at another hospital was nonspecific and the patient underwent surgical lung biopsy due to concern for interstitial lung disease. She subsequently became hemodynamically unstable and was transferred to our hospital where she presented with severe hypoxemia, hypotension, and suprasystemic pulmonary artery pressures. Preliminary lung biopsy results suggested idiopathic pulmonary arterial hypertension and the patient was started on vasodilating agents, including continuous epoprostenol infusion. Pulmonary artery pressures decreased but remained suprasystemic and the patient did not improve. Final review of the biopsy by a specialized laboratory revealed a diagnosis of PVOD after which vasodilating therapy was immediately weaned off. Evaluation for dual heart-lung transplantation was begun. The patient's hospital course was complicated by hypotension requiring vasopressors, worsening right ventricular dysfunction, and acute kidney injury. During the transplantation evaluation, the patient decided that she did not want to undergo continued attempts at stabilization of her progressive multi-organ dysfunction and she was transitioned to comfort care. She expired hours after removing inotropic support.

Management of Pulmonary Arterial Hypertension in the ICU

“…2 The use of PAH-specific therapies in patients with pulmonary veno-occlusive disease (PVOD) requires careful monitoring and is often avoided due to an increased incidence of pulmonary edema due to dilation of the pulmonary arteries thus worsening hypoxemia. 19,20 According to the joint European Society of Cardiology and the European Respiratory Society guidelines and the sixth World Symposium on Pulmonary Hypertension, after a confirmatory diagnosis of PAH, patients with idiopathic PAH should undergo vasoreactivity testing. 13,21 Vasoreactivity testing uses a short-acting pulmonary vasodilator such as intravenous (IV) epoprostenol, inhaled nitric oxide, inhaled iloprost, or IV adenosine to determine whether patients have an acute decrease in mPAP before committing patients to long-term treatment.…”

“…2 The use of PAH-specific therapies in patients with pulmonary veno-occlusive disease (PVOD) requires careful monitoring and is often avoided due to an increased incidence of pulmonary edema due to dilation of the pulmonary arteries thus worsening hypoxemia. 19,20…”