Pulmonary hypertension–related dynamic left ventricular outflow tract obstruction

A 62-year-old woman was admitted for progressive dyspnoea and oedema. She had been diagnosed with autoimmune hepatitis 10 years earlier. Transthoracic echocardiography (TTE) showed severe tricuspid regurgitation and pulmonary hypertension (PH) with an estimated systolic pressure of 170 mmHg (Panel A). Left ventricular wall thickness and systolic function were normal. Colour Doppler revealed turbulence in the left ventricular outflow tract (LVOT) with a peak pressure gradient of 86 mmHg (Panels B and C). Systolic anterior motion of the mitral valve was noted. Computed tomographic angiography excluded pulmonary embolism and intra-cardiac abnormalities and corroborated echocardiographic findings by demonstrating markedly dilated pulmonary artery and right heart and protrusion of the basal segment of the inter-ventricular septum towards the LVOT (Panels D and E), which explained the mechanism of LVOT obstruction (Panel F and Supplementary data online, Video S1). She was treated with oxygen, methylprednisolone, furosemide, spironolactone, and ambrisentan. Repeated TTE 1 week later showed improved PH and resolution of LVOT obstruction (Panels G–I). Myocardial contrast echocardiography excluded ventricular hypertrophy (see Supplementary data online, Videos S2 and S3). She was discharged with significantly relieved symptoms but unfortunately died of sudden death 2 weeks later. LVOT obstruction has been generally associated with hypertrophic cardiomyopathy. Dynamic LVOT obstruction related to PH is rare but may carry a very poor prognosis. In such a setting, an increase of pulmonary artery pressure and right ventricular overload could lead to decreased left ventricular filling and significant septal protrusion towards LVOT and result in suddenly worsened LVOT obstruction and death. Therefore, aggressive management of PH should be considered for those patients. Supplementary data are available at European Heart Journal online. The work was supported by Sichuan Science and Technology Program (Grant Number 2022ZDZX0030). All authors declare no conflict of interest for this contribution. The data underlying this article will be shared on reasonable request to the corresponding author.