Hipertensión pulmonar y enfermedad del tejido conectivo, una grave presentación

Abstract

Introduction

Pulmonary arterial hypertension (PAH) is a rare condition characterized by elevated pulmonary artery pressure (>20 mmHg) and increased vascular resistance. It affects 5 to 15 adults per million, causing dyspnea, chest pain, risk of right heart failure and death. Below, we describe a case of severe pulmonary arterial hypertension whose diagnosis was established after a recovered cardiac arrest.

Clinical Case

A 48-year-old man presented with edema, presyncope, and progressive dyspnea over 6 months. He suffered a cardiorespiratory arrest due to pulseless electrical activity and subsequent asystole, requiring resuscitation, norepinephrine, and mechanical ventilation. Echocardiography revealed severe pulmonary hypertension (pulmonary artery systolic pressure of 59 mmHg), preserved left ventricular function, and severe right ventricular dilation. Computed tomography angiography ruled out pulmonary embolism. Treatment with dobutamine and negative fluid balance improved his hemodynamics. Right heart catheterization confirmed precapillary pulmonary hypertension. Sildenafil, nitric oxide, and later, ambrisentan were initiated. Rheumatologic studies identified rheumatoid arthritis with overlapping Sjögren syndrome as the likely etiology of his pulmonary hypertension. The patient improved with targeted pulmonary hypertension therapy and prednisone, was successfully weaned off mechanical ventilation and was discharged, remaining stable at the one-month follow-up.

Discussion

PAH associated with connective tissue diseases, such as Sjögren syndrome and rheumatoid arthritis is rare. Treatments include endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostanoids, and drug combinations. Appropriate immunological treatment for underlying rheumatologic diseases, like rheumatoid arthritis, may improve PAH. In this case, an integral approach, including pulmonary vasodilators and preload control, was key to the patient's recovery.