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Impact of short-term high altitude exposure on exercise capacity and symptoms in Fontan patients.

Heart. 2016 08 15;102(16):1255-6

Authors: Brida M, Diller GP

PMID: 27230020 [PubMed - indexed for MEDLINE]

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Patients with Down syndrome and congenital heart disease: survival is improving, but challenges remain.

Heart. 2016 10 01;102(19):1515-7

Authors: Dimopoulos K, Kempny A

PMID: 27354276 [PubMed - indexed for MEDLINE]

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Genotype-phenotype correlations in Marfan syndrome.

Heart. 2017 11;103(22):1750-1752

Authors: Landis BJ, Veldtman GR, Ware SM

PMID: 28596305 [PubMed - indexed for MEDLINE]

Subvalvular aortic stenosis: a review of current literature.

Clin Cardiol. 2018 Jan 29;:

Authors: Devabhaktuni SR, Chakfeh E, Malik AO, Pengson JA, Rana J, Ahsan CH

Subvalvular aortic stenosis (SAS) is one of the common adult congenital heart diseases, with a prevalence of 6.5%. It is usually diagnosed in the first decade of life. Echocardiography is the test of choice to diagnose SAS. Surgical correction is the best treatment modality, and the prognosis is usually excellent. In this review, we describe the pathophysiology, diagnosis, prognosis, and management of SAS with a focus on different pathophysiologic mechanisms, diagnostic approach, and prognosis of the disease by reviewing the current literature.

PMID: 29377232 [PubMed - as supplied by publisher]

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Autoimmune Disease with Cardiac Valves Involvement: Libman-Sacks Endocarditis.

Acta Med Indones. 2017 Apr;49(2):148-150

Authors: Ginanjar E, Yulianto Y

This case study aim to evaluate the response of steroid treatment for autoimmune endocarditis. Valvular heart disease is relatively rising in both congenital and acquired cases, but the autoimmune endocarditis remains rare. In this case, a 34 year old woman with clinical manifestation resembling systemic lupus erythematosus (SLE) is diagnosed with Libman-sacks Endocarditis. After six months of steroid treatment, her clinical manifestations and heart structure improved.

PMID: 28790229 [PubMed - indexed for MEDLINE]

Systemic Right Ventricle in Adults With Congenital Heart Disease: Anatomic and Phenotypic Spectrum and Current Approach to Management.

Circulation. 2018 Jan 30;137(5):508-518

Authors: Brida M, Diller GP, Gatzoulis MA

The systemic right ventricle (SRV) is commonly encountered in congenital heart disease representing a distinctly different model in terms of its anatomic spectrum, adaptation, clinical phenotype, and variable, but overall guarded prognosis. The most common clinical scenarios where an SRV is encountered are complete transposition of the great arteries with previous atrial switch repair, congenitally corrected transposition of the great arteries, double inlet right ventricle mostly with previous Fontan palliation, and hypoplastic left heart syndrome palliated with the Norwood-Fontan protocol. The reasons for the guarded prognosis of the SRV in comparison with the systemic left ventricle are multifactorial, including distinct fibromuscular architecture, shape and function, coronary artery supply mismatch, intrinsic abnormalities of the tricuspid valve, intrinsic or acquired conduction abnormalities, and varied SRV adaptation to pressure or volume overload. Management of the SRV remains an ongoing challenge because SRV dysfunction has implications on short- and long-term outcomes for all patients irrespective of underlying cardiac morphology. SRV dysfunction can be subclinical, underscoring the need for tertiary follow-up and timely management of target hemodynamic lesions. Catheter interventions and surgery have an established role in selected patients. Cardiac resynchronization therapy is increasingly used, whereas pharmacological therapy is largely empirical. Mechanical assist device and heart transplantation remain options in end-stage heart failure when other management strategies have been exhausted. The present report focuses on the SRV with its pathological subtypes, pathophysiology, clinical features, current management strategies, and long-term sequelae. Although our article touches on issues applicable to neonates and children, its main focus is on adults with SRV.

PMID: 29378757 [PubMed - in process]

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Gender-specific care for adults with congenital heart disease: A look in the future?

Int J Cardiol. 2017 10 15;245:141-142

Authors: Drakopoulou M, Brida M

PMID: 28874284 [PubMed - indexed for MEDLINE]

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International collaborative initiative towards improving the lives of patients after the Fontan operation: A call for action.

Int J Cardiol. 2017 10 15;245:143-144

Authors: Brida M, Gatzoulis MA

PMID: 28874285 [PubMed - indexed for MEDLINE]

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[Three Surgical Cases of Quadricuspid Aortic Valve].

Kyobu Geka. 2017 Sep;70(10):811-815

Authors: Motohashi Y, Katsumata T, Suzuki T, Shimada R, Konishi H, Fukuhara S, Kanki S, Daimon M, Ozawa H, Nemoto S

Quadricuspid aortic valve is a rare congenital disease. We experienced 3 surgical cases of quadricuspid aortic valve. Patient 1 was a 72-year-old man who was noted to have a quadricuspid aortic valve associated with aortic regurgitation and an ascending aortic aneurysm(51 mm in diameter). He underwent replacement of the aortic valve and the ascending aorta. Patient 2 was a 71-year-old man with severe aortic stenosis, regurgitation, and coronary triple vessel disease. He underwent aortic valve replacement and coronary artery bypass grafting. Preoperative echocardiography revealed no abnormalities in the number of valve leaflets, but quadricuspid aortic valve was identified during surgery. Patient 3 was a 79-year-old man with severe aortic regurgitation, who underwent aortic valve replacement. In all patients, the 4 valve cusps were approximately of the same size. Multi-detector computed tomography is useful for evaluation of valve morphology. Indication of prophylactic ascending aorta replacement in patients with aortic dilatation requires further study.

PMID: 28894052 [PubMed - indexed for MEDLINE]

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Double-chambered left ventricle: coronary embolism as the first presentation of an extremely unusual cardiac anomaly.

J Cardiovasc Med (Hagerstown). 2017 10;18(10):790-792

Authors: Dallaglio PD, Claver E, Di Marco A, Alió J, Hidalgo A, Cequier A

PMID: 23549276 [PubMed - indexed for MEDLINE]