B60-21 Exertional Dyspnea: Is It the Heart, the Lungs, or the Vessels in Between?

Abstract Introduction Cough and shortness of breath are commonly attributed to asthma in children, but progressive respiratory or systemic symptoms should prompt further workup. We present a child with chronic cough and exertional dyspnea who initially improved with asthma therapy but ultimately worsened and was diagnosed with severe pulmonary arterial hypertension (PAH) and right heart failure. We suspect her initial symptoms of airflow obstruction were related to her pulmonary vasculature disease. Description A 4-year-old female with a family history of asthma and recent hospitalization for pneumonia presented to Pulmonary clinic with chronic dry cough for one year, triggered by respiratory infections, exertion, scents, smoke, and cats. She was trialed on inhaled corticosteroids and bronchodilators for suspected asthma with temporary improvement. Eight months later, family reported progressive shortness of breath, leg pain, and fatigue with daily activities, plus 3 weeks of abdominal pain and weight loss. Repeat chest radiograph (Figure 1A) in Pulmonary clinic revealed new cardiomegaly and enlarged central pulmonary arteries. She presented to the Emergency Department where exam was notable for a new murmur and hepatomegaly. Echocardiogram demonstrated suprasystemic right-sided pressure, bowing of the ventricular septum, severe right ventricular dilatation, and severe right ventricular dysfunction. Brain natriuretic peptide was elevated to 3034 pg/mL. She was admitted to the Cardiac Intensive Care Unit for severe pulmonary arterial hypertension (PAH) and right heart failure. Management involved supplemental oxygen, inhaled nitric oxide, and dopamine, followed by uptitration of targeted pulmonary vasodilator therapies (treprostinil, ambrisentan, and tadalafil). Cardiac catheterization 4 weeks later confirmed hemodynamic diagnosis of severe PAH (mean pulmonary artery pressure 85 mmHg). Genetic testing revealed a de novo BMPR2 mutation. Chest CT scan demonstrated interstitial lung disease (ILD) (Figure 1B). She started on sotatercept (novelactivin-signaling inhibitor). Cardiac dysfunction improved, though PAH remained severe. She was discharged after 2 months. Discussion PAH is an extremely rare cause of dyspnea, especially in children, and can lead to significant morbidity and mortality. Airflow obstruction has been observed in patients with PAH and is hypothesized to occur due to a common signal driving inflammation, smooth muscle contraction, and remodeling in the small airways and pulmonary vasculature. It is possible that the child’s asthma-like respiratory symptoms were related to her underlying PAH and BMPR2-related ILD, explaining initial response to albuterol and steroids. However, systemic signs including exercise intolerance, weight loss, and hepatomegaly warranted broadening of the differential diagnosis and additional workup for cardiac and other causes. This abstract is funded by: None