A 14-year-old boy presents with exertional dyspnea, clubbing, and central cyanosis that worsens over two years. He was diagnosed with a large VSD at birth but parents declined surgery. ECG shows right axis deviation and right ventricular hypertrophy. SpO2 is 82% on room air. The MOST important prognostic implication of this presentation is:

• A Surgical repair now will completely reverse pulmonary hypertension
• B Polycythemia from chronic hypoxia will protect against paradoxical embolism
• C Pulmonary vascular resistance has likely exceeded systemic resistance, making surgical repair contraindicated ✓
• D Cyanosis is due to development of concurrent ASD, and closure of ASD will resolve symptoms

Explanation

Eisenmenger syndrome occurs when a large left-to-right shunt causes progressive obliterative pulmonary vascular disease, eventually reversing the shunt direction (right-to-left) when pulmonary vascular resistance (PVR) exceeds systemic vascular resistance (SVR). At this stage, surgical repair is contraindicated because the hypertrophied right ventricle has become load-dependent on the shunt; closing the defect would cause acute right heart failure. Medical therapy (pulmonary vasodilators) and lung transplantation are the only options.

Reference: Ghai Essential Pediatrics, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.