Comparison of Angiotensin-Converting Enzyme Inhibitor (ACEI) and Angiotensin Receptor Blocker (ARB) for Heart Failure Treatment in Congenital Heart Diseases with Left-to-Right Shunt

BACKGROUND: The angiotensin-converting enzyme inhibitors (ACEIs) have become the forefront of heart failure treatment for more than a decade. Currently, angiotensin receptor blockers (ARBs) are thought to have similar effectiveness. This study aimed to compare the impact of captopril, one of ACEI, and valsartan, one of ARB, on clinical presentation and echocardiographic, electrocardiographic, and chest x-ray improvement in patients with left-to-right shunt congenitalheart diseases.METHODS: This study used a double-blind randomized controlled trial of captopril and valsatran to children with left-to-right shunt congenital heart diseases who suffer from heart failure in the Dr. Soetomo General Hospital, Surabaya, Indonesia. Pediatric heart failure scores, echocardiography, electrocardiography (ECG), and chest photographs were examined at the beginning of the study and after 30 days of treatment.RESULTS: A decrease in pediatric heart failure scores were showed after the administration of ACEI (7.06±2.04 vs. 4.75±2.43; p<0.0001; 95% CI: −2.98 - 1.65); ARB (6.81±2.25 vs. 3.94±1.98; p<0.0001; 95% CI: −3.76 to 1.98). The echocardiography examination, an increase in left ventricular end-diastolic volume (LVEDV), left ventricular ejection fraction (LVEF), % fractional shortening (FS), and left ventricular (LV) dimension occurred after the administration of ACEI and ARB. The values also didn’t significantly differrent between the two groups. The ECG evaluation showed a decrease in heart rate frequency after the administration of ACEI (117.75±14.67 vs. 109.63±17.59; p=0.039; 95% CI: −15.78 to −0.46) and ARB (117.10±21.86 vs.108.6±20.66; p=0.006; 95% CI: −14.17 to −2.83).CONCLUSION: ARB showed better outcome in clinical condition, echocardiography, ECG, and chest radiographs.KEYWORDS: captopril, valsartan, heart failure, congenital heart disease, left to right shunt

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Ebstein anomaly, left ventricular hypertrabeculation and ventricular septal defect associated with a myh7 mutation

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeaa356.006 ◽

2021 ◽

Vol 22 (Supplement_1) ◽

Author(s):

B Korkmaz ◽

U Aydogdu ◽

D Inan ◽

N Keles ◽

O Yildirimturk

Keyword(s):

Heart Failure ◽

Ventricular Septal Defect ◽

Medical Treatment ◽

Congenital Heart ◽

Septal Defect ◽

Heart Diseases ◽

Left Ventricular ◽

Congenital Heart Diseases ◽

Splice Acceptor ◽

Ebstein Anomaly

Abstract Funding Acknowledgements Type of funding sources: None. Ebstein anomaly is an extremely rare anomaly of <1% among all congenital heart diseases. Pathologically, the septal and / or posterior leaflet of the tricuspid valve has abnormal locations towards the right ventricular apex. Ebstein anomaly is especially accompanied by atrial septal defect, patent ductus arteriosus, wolf parkinson white syndrome and pulmonary atresia. Defects located in the interventricular septum are called the ventricular septal defect (VSD). They can be single or multiple and congenital or acquired. Isolated VSDs are the most common congenital anomaly in childhood and constitute 20-30% of all congenital heart diseases. VSD can be a part of major congenital malformations ,such as, fallot tetralogy, transposition of the major arteries, double ventricular right ventricle. Left ventricular noncompaction (LVNC) is a relatively common genetic cardiomyopathy, characterized by prominent trabeculations with deep intertrabecular recesses in mainly the left ventricle. Although LVNC often occurs in an isolated entity, it may also be present in various types of congenital heart disease . A combination of Ebstein anomaly, hypertrabeculation and ventricular septal defect is a rare condition. Case Report A 49-year-old male patient presented to the emergency room with shortness of breath and swelling of the legs. The patient had diagnosed an Ebstein anomaly while the military examination in 1988. Already it ‘s known that he has gout disease and uses colchicine but no family history of any disease. On examination of the patient, bilateral ral in respiratory sounds and +++ / +++ pretibial edema in the lower extremity were detected. On his electrocardiogram, the sinus rhythm with first-degree atrioventricular block was observed. The findings on his echocardiographic examination are ejection fraction 30-35% with global left ventricular hypokinesia, Ebstein anomaly (Figure ), perimembranous type VSD, atrial septal aneurysm type 2 and left ventricular hypertrabeculation. His blood table was normal. Medical treatment of heart failure was started for the patient who was interneed to the service. After getting clinical relief, the patient was discharged under medical treatment. Genetic tests were studied while following up at the heart failure outpatient clinic. In the MYH7 gene, splice-acceptor-2 (PVS1) variation heterozygous was detected. This variant has not been seen in national data banks of genetics. Conclusion The MYH7 gene, localized on chromosome 14p12, is composed of 41 exons and encodes the b-myosin heavy chain, expressed in cardiac muscle. Mutations in the MYH7 gene have been identified in association with left ventricular hypertrabeculation and Ebstein anomaly. In conclusion, this is the first known report of Ebstein anomaly associated with the splice-acceptor-2 variation heterozygous of the MYH7 gene. Abstract Figure

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