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A haemodynamic conundrum: a case report of a patient with concurrent pulmonary arterial hypertension and hypertrophic obstructive cardiomyopathy.

ABSTRACT:

Background

The co-existence of hypertrophic obstructive cardiomyopathy (HOCM) and pulmonary arterial hypertension (PAH) is extremely rare and poses a management conundrum. This is the first case report in the published literature to describe the diagnosis and management of a patient with both conditions.

Case summary

A 49-year-old female with a history of HOCM and recently diagnosed scleroderma presented to the clinic with progressive dyspnoea. Transthoracic echocardiogram demonstrated left ventricular outflow tract (LVOT) obstruction at rest, and elevated pulmonary artery (PA) pressure. Cardiac catheterization (CC) demonstrated an LVOT gradient of 150 mmHg, PA pressure of 88/32 mmHg, pulmonary capillary wedge pressure (PCWP) 12 mmHg, pulmonary vascular resistance 14.8 Wu, and a cardiac index of 1.6 L/min/m². The differential diagnosis for the dyspnoea included combined pre- and post-capillary pulmonary hypertension from longstanding HOCM vs. scleroderma associated PAH. Tadalafil was added to the patient's medical regimen of metoprolol but it was stopped because the patient developed pulmonary oedema. Alcohol septal ablation was undertaken with improvement in the LVOT gradient, but only a modest improvement in her dyspnoea. Repeat CC demonstrated worsening PAH. Vasodilatory testing with nitric oxide led to an improvement in the PA pressure with minimal increase of the PCWP. Hence, she was started on treprostinil and macitentan, with significant improvement in her dyspnoea on follow-up.

Conclusion

In patients with concurrent HOCM and advanced PAH, a multidisciplinary treatment approach is needed to rapidly and safely optimize the background of HOCM in order to permit the use of PAH-specific medications.

SUBMITTER: Hassanin A

PROVIDER: S-EPMC8453412 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:BackgroundThere are limited data on the prevalence, pathophysiology, and management implications of pulmonary hypertension in patients with obstructive hypertrophic cardiomyopathy and advanced heart failure.Methods and resultsTo assess the clinical significance of measured cardiopulmonary hemodynamics in hypertrophic cardiomyopathy patients with heart failure, we retrospectively assessed right heart catheterization data in 162 consecutive patients with outflow tract gradients (median [interquartile range], 90 mm Hg [70-110 mm Hg]), 59±11 years old, and 49% men, predominately New York Heart Association class III/IV status. Pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg) was present in 82 patients (51%), including 29 (18%) regarded as moderate-severe (mean pulmonary artery pressure, ≥35 mm Hg) and 28 (34%) also had increased pulmonary vascular resistance >3.0 WU. The pulmonary artery wedge pressure was ≤15 mm Hg in 54%, indicating that left atrial hypertension was absent in a majority of patients. Notably, 9 patients (11%) met hemodynamic criteria for precapillary pulmonary hypertension (mean pulmonary artery pressure, ≥25 mm Hg; pulmonary vascular resistance, >3.0 WU; pulmonary artery wedge pressure, ≤15 mm Hg). Over a median follow-up of 327 days (90-743 days) after surgical myectomy (or alcohol septal ablation), 92% and 95% of patients with or without preoperative pulmonary hypertension, respectively, were asymptomatic or mildly symptomatic. One postoperative death occurred in a 59-year-old woman with acute respiratory failure and mean pulmonary artery pressure of 65 mm Hg.ConclusionsPulmonary hypertension was common in obstructive hypertrophic cardiomyopathy patients with advanced heart failure. Although possibly a contributor to preoperative heart failure, pulmonary hypertension did not significantly influence clinical and surgical outcome. Notably, a novel patient subgroup was identified with resting invasive hemodynamics consistent with pulmonary vascular disease.