Abstract Introduction Pulmonary veno-occlusive disease (PVOD) is a rare and life-threatening cause of pulmonary arterial hypertension (PAH) with an estimated prevalence of 1 case per 1 million. Due to its rarity and diagnostic complexity, PVOD remains a challenging entity requiring a high index of suspicion and multidisciplinary evaluation. Early recognition is critical, as lung transplantation remains the only definitive treatment. This case illustrated the diagnostic and therapeutic challenges of PVOD in a patient with multifactorial pulmonary hypertension and underscores the importance of individualized management and timely diagnosis. Description A 67-year-old man with hypertension, diabetes, obesity class II, emphysema, and severe central/obstructive sleep apnea was sent to the emergency department from his pulmonology clinic for significant hypoxia (SpO2 86%) and worsening dyspnea. Chest X-ray showed mild interstitial edema. CTA ruled out pulmonary embolism but demonstrated diffuse centrilobular ground-glass opacities, interlobular septal thickening, and mediastinal lymphadenopathy, suggestive of PVOD. Echocardiogram showed a dilated right atrium and right ventricle with pulmonary artery systolic pressure (PASP) of 85 mmHg, prompting initiation of diuretics. Right heart catheterization (RHC) showed a mean pulmonary artery pressure (MPAP) of 38 mmHg and a pulmonary vascular resistance (PVR) of 9 wood units (WU). Extensive serologic workup was negative. Following BiPAP initiation for severe sleep apnea, repeat RHC after one month showed worsening MPAP (65 mmHg) and PVR (13.5 WU). He was started on low-dose tadalafil and ambrisentan, later uptitrated with close monitoring. Despite an episode of vasodilator-induced pulmonary edema requiring dose adjustment, he showed marked clinical and echocardiographic improvement, was weaned off supplemental oxygen, and deferred lung transplant evaluation due to significant recovery in function and quality of life. Discussion Pulmonary veno-occlusive disease (PVOD) is progressive with poor prognosis, making timely recognition and management critical. Response to PAH-directed therapy is highly variable; some patients achieve meaningful improvement, while others may develop pulmonary edema or remain refractory. Recent data illustrate that carefully titrated vasodilators, balanced with diuretics and close monitoring, can benefit select patients, though long-term effects are variable and remain poorly understood. In our patient, this approach, combined with optimization of comorbidities, resulted in significant clinical and hemodynamic improvement. This case emphasizes the need for individualized care in complex PVOD and the importance of ongoing research to define which patients benefit from PAH-directed therapy and clarify its long-term safety and efficacy. This abstract is funded by: None
Umra et al. (Fri,) studied this question.
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