Author + information
- Lakshmi Meyyappan,
- Amanda Wasserman,
- Julian Neshewat,
- Susanne McDevitt,
- Victor Moles and
- Vallerie V. McLaughlin
Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis (PCH) are rare causes of pulmonary hypertension (PH) and occur in about 1 per 10 million people. Diagnosing PVOD/PCH is challenging since patients commonly present with symptoms similar to pulmonary arterial hypertension (PAH), leading to a delay in a definitive diagnosis. PVOD/PCH represent 5–10% of the cases initially thought to be idiopathic PAH. Because PVOD/PCH is a rare form of PAH, this population is not well described in literature. Understanding clinical differences at the time of diagnosis of PAH may help to earlier differentiate PVOD/PCH from idiopathic PAH.
We searched our University of Michigan Pulmonary Hypertension database for patients with PAH and PVOD/PCH from 2016–2018. Data at the time of PH diagnosis was abstracted for both populations, including baseline testing results for brain natriuretic peptide (BNP), echocardiogram, right heart catheterization, pulmonary function tests (PFT), and 6-minute hall walk (6MW).
127 PAH and 25 PVOD/PCH patients were identified. On average, the time from PAH diagnosis to a presumed clinical PVOD/PCH diagnosis was 2.85 years. At baseline, more PVOD/PCH patients were classified as WHO functional class III or IV (80% v. 63.8%; p=0.033). These patients had a higher BNP (490.9 ± 138.4 v. 283.4 ± 41.4; p=0.007), more severe RV dysfunction (TAPSE 15.73 ± 1.48 v. 20.07 ± 0.89; p=0.018), and more advanced hemodynamics – including lower cardiac index (2.2 ± 0.14 v. 2.56 ± 0.09; p<0.001) and higher mean PA pressure (49.1 ± 2.5 v. 42.9 ± 1.5; p<0.001) compared to PAH patients. The PVOD/PAH patients had a significantly lower DLCO (42.78 ± 4.54 v. 57.37 ± 2.69; p< 0.001) as well as lower resting (94.9 ± 1.1 v. 97.7 ± 0.3; p<0.001) and peak oxygen (87.2 ± 1.4 v. 92.9 ± 0.8; p<0.001) saturations during the 6MW.
Overall, PVOD/PCH patients presented with worse functional class, RV function, and hemodynamics than PAH patients. Moreover, the PFT and 6MW test results were significantly worse for PVOD/PCH patients. These results should raise clinical suspicion for PVOD/PCH at baseline given that these patients present worse than other PAH patients.
Moderated Poster Contributions
Congenital Heart Disease and Pulmonary Hypertension Moderated Poster Theater, Posters, Hall A Saturday, March 28, 2020, 12:30 p.m.-12:40 p.m.
Session Title: A Potpourri of Interesting Topics in Pulmonary Hypertension
Abstract Category: 35. Pulmonary Hypertension
Presentation Number: 1014-03
- 2020 American College of Cardiology Foundation