Pulmonary Vascular Disease |

Pulmonary Arterial Hypertension- 4 Causes, 1 Patient FREE TO VIEW

Navitha Ramesh, MD; David Nesheim, MD; Jason Filopei, MD; Roxana Sulica, MD
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Mount Sinai Beth Israel, Chesterfield, NJ

Chest. 2015;148(4_MeetingAbstracts):964A. doi:10.1378/chest.2279233
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SESSION TITLE: Pulmonary Vascular Disease Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: We present a unique case of APAH(associated pulmonary arterial hypertension) in which the patient four had different etiologic risk factors.

CASE PRESENTATION: A 52-year-old woman with HIV and portal hypertension (esophageal varices) due to hepatitis C-cirrhosis, presented in hypoxic respiratory failure, requiring non-invasive ventilation. Lungs were clear to auscultation, she had 2+ pitting lower extremity edema and ascites, and a loud second heart sound. At baseline she had WHO functional class III symptoms. Transthoracic echocardiogram with bubble study demonstrated a right-to-left interatrial shunt, estimated pulmonary artery systolic pressure of 117 mmHg, dilated right atrium and ventricle with hypokinetic right ventricle. Cardiac MRI showed a defect along the inter-atrial septum measuring 13 mm. The patient was not aware of the atrial septal defect diagnosis. Ventilation/perfusion scan was normal. Serum studies revealed elevated rheumatoid factor and elevated anti-CCP antibody, and rheumatologic evaluation confirmed the diagnosis of rheumatoid arthritis. Right heart catheterization performed after diuresis and resolution of hypoxemia showed mean pulmonary artery pressure of 48 mmHg, right atrial pressure of 5 mmHg, pulmonary artery wedge pressure of 4 mmHg, cardiac index of 2.18 L/min/m2 and pulmonary vascular resistance of 13.96 Wood units. Upon discharge she was started on Macitentan and Riociguat in rapid succession. Three months later her functional status improved to functional class II and the distance walked during 6 minutes increased by 38 m.

DISCUSSION: Pulmonary arterial hypertension (PAH) is a chronic, progressive disease leading to right heart failure and ultimately death if untreated. Early recognition and treatment are important. In general, patients with APAH present with one known condition that leads to the development of the disease. We have found four different conditions in our patient: HIV infection, connective tissue disease, porto-pulmonary hypertension, and congenital heart disease. The accurate diagnosis of pulmonary hypertension requires a battery of tests to screen for other potential associated conditions that may post-date the diagnosis of PAH.

CONCLUSIONS: The work-up of PAH patients should diligently follow published guidelines. Even in patients with multiple pathogenetic pathways and severe pulmonary hypertension, specific PAH therapy may lead to positive outcomes.

Reference #1: Hoeper MM et al. Definitions and Diagnosis of Pulmonary Hypertension. JACC 2013; 62:D42-50

DISCLOSURE: The following authors have nothing to disclose: Navitha Ramesh, David Nesheim, Jason Filopei, Roxana Sulica

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