ABSTRACT
d-loop transposition of the great arteries (d-TGA) and congenitally corrected transposition of the great arteries (cc-TGA) feature a right ventricle attempting to sustain the systemic circulation. A systemic right ventricle (sRV) cannot support cardiac output in the long run, eventually decompensating and causing heart failure. The burden of d-TGA patients with previous atrial switch repair and cc-TGA patients with heart failure will only increase in the coming years due to the aging adult congenital heart disease population and improvements in the management of advanced heart failure. Clinical data still lags behind in developing evidence-based guidelines for risk stratification and management of sRV patients, and clinical trials for heart failure in these patients are underrepresented. Recent studies have provided foundational data for the commencement of robust clinical trials in d-TGA and cc-TGA patients. Further insights into the multifactorial nature of sRV failure can only be provided by the results of such studies. This review discusses the mechanisms of heart failure in sRV patients with biventricular circulation and how these mediators may be targeted clinically to alleviate sRV failure.
ABSTRACT
No disponible
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Heart Septal Defects, Ventricular/urine , Hypertension, Pulmonary/surgery , Angioplasty, Balloon/methods , Retrospective Studies , Treatment Outcome , Pulmonary Wedge Pressure/physiologySubject(s)
Atrial Septum/surgery , Cardiac Surgical Procedures/methods , Hypertension, Pulmonary/surgery , Pulmonary Wedge Pressure/physiology , Atrial Septum/diagnostic imaging , Cardiac Catheterization , Child , Child, Preschool , Echocardiography , Female , Follow-Up Studies , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/physiopathology , Infant , Male , Retrospective StudiesABSTRACT
A newborn was referred to our department, due to the presence of a cardiac murmur. Echocardiographic study showed a small membranous ventricular septal defect and a yuxta-aortic mass. In the follow-up, patient remained asymptomatic, ventricular septal defect closed, and yuxta-aortic mass increase in size with somatic growth. A transesophageal echocardiography was carried out, showing a fibrotic and dyskinetic left ventricular subaortic aneurysm of 19×18 mm. Given that the patient remained asymptomatic, a conservative approach was adopted. Congenital left ventricular subaortic aneurysm is an infrequent entity, and rupture, endocarditis, coronary compression, thromboembolism, arrhythmias and aortic insufficiency are some of its complications, but, as in our patient, if aneurysm is not associated with complications, patient is asymptomatic, and aneurysm growth is not very important, a conservative approach can be adopted, with a close surveillance.
Subject(s)
Heart Aneurysm/diagnostic imaging , Heart Septal Defects, Ventricular/diagnostic imaging , Child , Female , Heart Aneurysm/congenital , Heart Septal Defects, Ventricular/complications , Humans , UltrasonographyABSTRACT
A 29-week-old male foetus was diagnosed by foetal echocardiography with severe hypertrophic cardiomyopathy with systolic dysfunction and generalized oedema, undergoing a Caesarean section at 33 weeks. Mechanical ventilation and milrinone infusion were required during the first week. Systolic function and output parameters improved progressively. Metabolic and infectious screenings were negative. At the follow-up, during the first year of life, hypertrophy regressed, posterior right auricular hypertrophy evolved to a mass with cysts, and left ventricular myocardium developed trabeculations accomplishing non-compaction criteria. Recently, mutations in genes previously implicated in the pathogenesis of hypertrophic cardiomyopathy have been identified in patients with left ventricular non-compaction without hypertrophy. This report suggests that these cardiomyopathies may have a similar genetic origin, and can co-exist in the same patient.
