A haemodynamic conundrum: a case report of a patient with concurrent pulmonary arterial hypertension and hypertrophic obstructive cardiomyopathy.
Alcohol septal ablation
Haemodynamics
Hypertrophic cardiomyopathy
Pulmonary hypertension
Journal
European heart journal. Case reports
ISSN: 2514-2119
Titre abrégé: Eur Heart J Case Rep
Pays: England
ID NLM: 101730741
Informations de publication
Date de publication:
Sep 2021
Sep 2021
Historique:
received:
03
02
2021
revised:
19
03
2021
accepted:
23
08
2021
entrez:
24
9
2021
pubmed:
25
9
2021
medline:
25
9
2021
Statut:
epublish
Résumé
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. 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/m 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.
Sections du résumé
BACKGROUND
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
METHODS
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/m
CONCLUSION
CONCLUSIONS
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.
Identifiants
pubmed: 34557642
doi: 10.1093/ehjcr/ytab354
pii: ytab354
pmc: PMC8453412
doi:
Types de publication
Case Reports
Langues
eng
Pagination
ytab354Informations de copyright
© The Author(s) 2021. Published by Oxford University Press on behalf of the European Society of Cardiology.
Références
J Am Coll Cardiol. 2013 Dec 24;62(25 Suppl):D51-9
pubmed: 24355642
Am J Cardiol. 2007 Oct 15;100(8):1293-8
pubmed: 17920373
Ann Rheum Dis. 2013 Dec;72(12):1940-6
pubmed: 23178295
Int J Cardiol. 2017 Dec 1;248:326-332
pubmed: 28733069
J Am Coll Cardiol. 2015 Mar 31;65(12):1249-1254
pubmed: 25814232
Ann Rheum Dis. 2014 Jul;73(7):1340-9
pubmed: 23687283
Circ Heart Fail. 2017 Apr;10(4):e003689
pubmed: 28396501
Eur Heart J Cardiovasc Imaging. 2016 Jun;17(6):604-10
pubmed: 26922089
Am J Cardiol. 2003 Apr 1;91(7):817-21
pubmed: 12667567