Abstract Introduction Pulmonary Arterial Hypertension (PAH) is a devastating disease that can affect young women. It is relatively rare. Delayed diagnosis can lead to Right heart failure and death. Patients with PAH carry a high mortality risk associated with surgery. Uterine fibroids are common in young women as well. We present a case of a 28-year-old young female who presented with a large uterine leiomyoma and severe PAH simultaneously. Case Presentation A 28-year-old female presented with worsening shortness of breath which was abrupt in onset along with a large abdominal pelvic mass. Pertinent signs and symptoms included bilateral lower extremity pitting edema, dizziness on bending, bendopnea. She denied any pulmonary vascular risk factors such as VTE, connective tissue disease or recreational drug use. Echocardiogram showed severely dilated right atrium and right ventricle with interatrial septal bowing towards the left atrium. The estimated RVSP was 70.5 mmHg. Right heart catheterization confirmed WHO Group I PAH with FC III - Intermediate to high-risk features. A CT abdomen showed a large conglomerate mass arising from the uterus and/or adnexa measuring 33 x 20 x 20 cm, with 16 smaller masses, the largest one measuring 26 cm in diameter. CT Pulmonary angiogram was negative for pulmonary embolism but revealed enlargement of the main pulmonary artery. In discussion with OBGYN, surgery was recommended as soon as possible. A semi-elective surgery was planned. Early combination therapy with tadalafil 40 mg and macitentan 10 mg daily resulted in an excellent clinical response in the short term. She was electively admitted 3 days ahead of planned surgery and started on parenteral prostacyclin therapy (PPT). She underwent resection of the large uterine fibroid with a successful outcome. PPT was weaned off postoperatively. Figure 1 presents the patient’s right heart catheterization, pulmonary function tests, and laboratory results, and images of the removed mass. After successful surgery, she was started on Sotatercept and is doing well clinically as WHO FC-I. Conclusion This case illustrates concomitant presentation of PAH and large uterine leiomyoma in a young woman. A comprehensive systematic evaluation and a multidisciplinary approach resulted in a favorable outcome. This abstract is funded by: None
Javed et al. (Fri,) studied this question.