Blow by Graham Steell

Specialty: Cardiovascular.

• Graham Steell diastolic murmur
• pulmonary insufficiency murmur due to pulmonary hypertension

Why it occurs

• Severe pulmonary arterial hypertension (primary or idiopathic)
• Chronic hypertensive pulmonary thromboembolism (Group 4)
• Chronic obstructive pulmonary disease (COPD) with severe cor pulmonale
• Advanced chronic left ventricular failure with severe passive pulmonary hypertension (Group 2)
• Chronic narrow mitral stenosis (classic cause described by Graham Steell)

Initial workup

Transthoracic Doppler echocardiogram to quantify the pulmonary artery systolic pressure (PSAP) using the tricuspid regurgitation gradient and the diastolic pulmonary regurgitation velocity (pulmonary diastolic pressure), measure the systolic excursion of the plane of the tricuspid annulus (TAPSE <17 mm indicates a poor prognosis) and assess the degree of dilation and systolic dysfunction of the right ventricle; Right heart catheterization for standard invasive measurement of pulmonary pressures, cardiac output, and pulmonary vascular resistance (PVR); Computed tomography (CT angiography) of the chest to rule out chronic pulmonary thromboembolism.

Standard management

• Treprostinil — prostacyclin analog indicated in severe pulmonary arterial hypertension to induce vasodilation of the pulmonary vasculature and reduce right ventricular afterload; Continuous subcutaneous or intravenous infusion adjusted according to pulmonary hypertension protocol
• Bosentan — dual endothelin receptor antagonist indicated to improve exercise tolerance and slow the progression of PAH; 62.5 to 125 mg twice daily orally, monitoring hepatic transaminases
• Sildenafil — phosphodiesterase-5 inhibitor indicated to promote nitric oxide-mediated vasodilation; 20 mg three times a day orally
• Furosemide (loop diuretic for the control of right ventricular volume overload and associated congestive hepatomegaly; 40 mg once daily orally, closely monitoring electrolyte balance).