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Cardiology / Cardiovascular

PAH - Congenital Heart Disease Associated

ICD-10 Code
I27.89_1

Clinical Criteria for PAH - Congenital Heart Disease Associated.

Clinical Presentation & Protocol

Patient Usually Complains Of

Patient presents for follow-up of PAH associated with congenital heart disease (CHD). Reports [stable/worsening] dyspnea on exertion, NYHA functional class [I/II/III/IV], and occasional palpitations. Denies syncope, chest pain, or hemoptysis. Current CHD anatomy: [e.g., ASD/VSD/PDA/Complex]. Adherent to PAH-specific therapy: [e.g., PDE5i/ERA/Prostacyclin].

Clinical Examination Findings

Vitals: BP [x/x], HR [x], SpO2 [x]% on [RA/O2]. General: No acute distress. CV: Prominent P2, right ventricular heave, holosystolic murmur at [left sternal border/apex] consistent with [shunt/regurgitation]. JVD present. Extremities: [1+/2+] pitting edema, no cyanosis or clubbing. Lungs: Clear to auscultation.

Treatment Protocol

Plan: Continue current PAH-targeted therapy: [Drug/Dose]. Monitor for side effects. Optimize fluid balance with [Diuretic]. Schedule repeat TTE and 6-minute walk test (6MWT) to assess functional status. Maintain close follow-up with congenital cardiology and pulmonary hypertension clinic.

Detailed clinical guide coming soon.