Abstract

Background: Twin pregnancies have a higher risk of congenital heart defects compared to singleton pregnancies. The most commonly found congenital heart defect in both monochorionic and dichorionic twin gestations is ventricular septal defect (VSD). Approximately 10% of individuals with congenital heart defects will develop pulmonary arterial hypertension-congenital heart disease (PH-CHD).  Case Presentation: We diagnosed an 11-year-old twin girl with ventricular septal defect. First Patient had a severe PH with mean Pulmonary Artery Pressure (mPAP) 68 and a defect size of 9-12 mm, while second patient had a mild PH with mPAP 30 and a defect size of 6-9 mm. The patient presented with failure to thrive, exercise intolerance during daily activities, and a history of recurrent respiratory tract infections. Echocardiography and right heart catheterization (RHC) results showed that the first patient with VSD Left to Right Shunt with low flow high resistance, non-reactive oxygen test and pulmonary hypertension. The second patient with VSD Let to Right shunt. Both of the patients were diagnosed with ventricular septal defect (VSD) and pulmonary hypertension (PH). However, they received different treatments based on the RHC results.  Conclusion: Promptly addressing the long-term implications of CHD is essential to prevent pulmonary hypertension (PH) and systemic disorders. Advanced PH treatments are safe and can result in improved hemodynamic, exercise capacity, quality of life, growth and development and potentially survival.

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