scholarly journals Left Ventricular Non-Compaction Cardiomyopathy, Ventricular Septal Defect and Pre-excitation: A Rare Coexistence of Three Abnormities in an Adult Patient.

2021 ◽  
Vol 18 (1) ◽  
pp. 57-59
Author(s):  
Tufan Çınar ◽  
Vedat Çiçek ◽  
Sahhan Kılıç ◽  
Emre Yalçınkaya ◽  
Murat Selçuk ◽  
...  

A congenital cardiac condition characterized by ventricular trabeculations and intertrabecular recesses is described as a non-compaction cardiomyopathy. In clinical practice, since it can be associated with severe mortality and morbidity due to progressive heart failure, thromboembolic events, and fatal arrhythmias, it is important to consider non-compaction cardiomyopathy. In this case, we have an adult patient who underwent surgery due to a ventricular septal defect (VSD) and who was later diagnosed with left ventricular non-compaction cardiomyopathy (LVNC) and pre-excitation. To our knowledge, this is the first case of VSD, LVNC, and pre-excitation simultaneously present in an adult patient.

2020 ◽  
Vol 36 (2) ◽  
pp. 159-165
Author(s):  
Hirotaka Ito ◽  
Hiroshi Suzuki ◽  
Kenichi Watanabe ◽  
Hisanori Haniu ◽  
Satoshi Hoshina ◽  
...  

2020 ◽  
Vol 30 (5) ◽  
pp. 743-745
Author(s):  
Selman Gokalp ◽  
Sezen Ugan Atik ◽  
Irfan L. Saltik

AbstractLeft ventricular pseudoaneurysm is very rare in children. Although surgery is conventional treatment, recently, percutaneous closure of pseudoaneurysms has been described. Here, we present the first case where a patient developed left ventricular pseudoaneurysm after percutaneous ventricular septal defect device closure and was treated by a second percutaneous method.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
B Korkmaz ◽  
U Aydogdu ◽  
D Inan ◽  
N Keles ◽  
O Yildirimturk

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


1995 ◽  
Vol 269 (4) ◽  
pp. H1473-H1480
Author(s):  
J. M. Stewart ◽  
T. H. Hintze ◽  
P. K. Woolf ◽  
M. S. Snyder ◽  
K. P. Seligman ◽  
...  

To assess the contributions of systolic and diastolic dysfunction to congestive heart failure (CHF) in ventricular septal defect (VSD), we studied 13 children with VSD at catheterization using a Millar catheter. Eight children had CHF, whereas five did not. Phenylephrine was infused at a rate of 5 micrograms.kg-1.min-1, and M-mode echocardiography and pressure were measured simultaneously. Systolic left ventricular (LV) function was assessed by maximum LV pressure (LVP), rate of pressure development (dP/dt), and by the end-systolic pressure-diameter relation (ESPDR). Systolic myocardial function was assessed by the end-systolic stress-strain relation. Diastolic chamber function was assessed by the isovolumic relaxation time constant (tau) and by the end-diastolic pressure-diameter relation (EDPDR). Diastolic myocardial function was measured by the end-diastolic stress-strain relationship. With phenylephrine, maximum LVP increased from 99 +/- 5 to 119 +/- 4 mmHg with CHF and from 106 +/- 6 to 149 +/- 10 mmHg without CHF. +dP/dt was lower with CHF (1,582 +/- 96 mmHg/s) than without CHF (2,300 +/- 200 mmHg/s). The maximum slope of the ESPDR was 39 +/- 8 with CHF and 94 +/- 14 mmHg/cm without CHF. The maximum slope of the midwall stress-strain relation was 223 +/- 37 with CHF and 395 +/- 93 g/cm2 without CHF. tau was 25 +/- 2 without CHF compared with 32 +/- 3 ms with CHF. The EDPDR was shifted leftward with failure, whereas the stress-strain relation was similar for all patients. CHF in patients with VSD results primarily from systolic dysfunction.(ABSTRACT TRUNCATED AT 250 WORDS)


2015 ◽  
Vol 72 (1) ◽  
pp. 68-71 ◽  
Author(s):  
Ljupco Mangovski ◽  
Rainer Kozlik-Feldmann ◽  
Miodrag Peric ◽  
Ljiljana Jovovic ◽  
Mihajlo Farkic ◽  
...  

Introduction. Acquired ventricular septal defect (VSD) is uncommon, but serious mechanical complication of acute myocardial infarction with poor outcome and high mortality rate in surgically or medically treated patients. Case report. We report a 58-year-old male patient admitted to our hospital six days following acute inferior myocardial infarction complicated by ventricular septal rupture with signs of heart failure. Coronary angiography revealed 3-vessel disease, with proximally occluded dominant right coronary artery. Transthoracic echo exam revealed aneurysm of a very thin inferior septum and the basal portion of the inferior left ventricular wall, with septal wall rupture. One of the VSD dimensions was 15 mm and left- to right shunt was calculated 2 : 1. Since the patient was at too high risk for surgical closure, transcatheter closure of VSD was chosen as a better option. Under short intravenous sedation, 24 mm Amplatzer device was implanted percutaneously with transesophageal echo guidance. The post-procedural result revealed a small residual shunt, but it was followed by significant improvement of the patient?s clinical status. A 24h Holter ECG monitoring did not show cardiac rhythm or conduction disturbances. Coronary angiography was repeated ten days following the procedure, after hemodynamic stabilization of the patient, with direct stenting of the circumflex artery and the intermediate artery. Ostial left descending artery lesion was left for further functional significance assessment. Conclusion: Percutaneous closure with a septal occluder device can be definitive primary treatment for anatomically suitable patients or it can serve as a bridge to surgical treatment.


2017 ◽  
Vol 2 (2) ◽  
pp. 69-74
Author(s):  
Mohammad Aminullah ◽  
Fahmida Akter Rima ◽  
Asraful Hoque ◽  
Mokhlesur Rahman Sazal ◽  
Prodip Biswas ◽  
...  

Background: Cardiac remodeling is important issue after surgical closure of ventricular septal defect.Objective: The purpose of the present study was to evaluate cardiac remodeling by echocardiography by measuring the ejection fraction, fractional shortening, left ventricular internal diameter during diastole (LVIDd) and left ventricular internal diameter during systole (LVIDs) after surgical closure of ventricular septal defect in different age group. Methodology: This prospective cohort studies was conducted in the Department of Cardiac Surgery at National Institute of Cardiovascular Disease (NICVD), Dhaka. Patient with surgical closure of VSD were enrolled into this study purposively and were divided into 3 groups according to the age. In group A (n=10), patients were within the age group of 2.0 to 6.0 years; age of group B (n=8) patients were 6.1-18.0 years and the group C (n=6) aged range was 18.1-42.0 years. Echocardiographic variables such as ejection fraction, fractional shortening, LVIDd, LVIDs were taken preoperatively and at 1st and 3rd month of postoperative values. Result: A total number of 24 patients was recruited for this study. The mean ages of all groups were 12.60±12.09. After 1 month ejection fraction were decreased by 5.97%, 6.71% and 5.66% in group A, group B and group C respectively. After 3 months ejection fraction were increased by 6.13%, 5.13% and 5.14% in group A, group B and group C respectively. After 1 month fractional shortening were decreased by 13.55%, 9.30% and 9.09% in group A, group B and group C respectively. After 3 months fractional shortening were increased by 7.23%, 7.35% and 4.55% in group A, group B and group C respectively. After 1 month LVIDd were increased by 1.97%, 1.91% and 1.32% in group A, group B and group C respectively. After 3 months LVIDd were decreased by 10.84%, 9.89% and 7.34% in group A, group B and group C respectively. After 1 month LVIDs were increased by 2.19%, 2.86% and 1.98% in group A, group B and group C respectively. After 3 months LVIDs were decreased by 11.68%, 10.97% and 8.87% in group A, group B and group C respectively.Conclusion: Cardiac remodeling occurred after surgical closure of ventricular septal defect and remodeling were more significant in younger age group. Journal of National Institute of Neurosciences Bangladesh, 2016;2(2):69-74


2021 ◽  
Vol 5 (5) ◽  
Author(s):  
Klaus-Dieter Hönemann ◽  
Steffen Hofmann ◽  
Frank Ritter ◽  
Gerold Mönnig

Abstract Background A rare, but serious, complication following transcatheter aortic valve replacement (TAVR) is the occurrence of an iatrogenic ventricular septal defect (VSD). Case summary We describe a case of an 80-year-old female who was referred with severe aortic stenosis for TAVR. Following thorough evaluation, the heart team consensus was to proceed with implantation via a transapical approach of an ACURATE neo M 25 mm valve (Boston Scientific, Natick, MA, USA). The valve was deployed harnessing transoesophageal echocardiographic (TOE) guidance under rapid pacing with post-dilation. Directly afterwards a very high VSD close to the aortic annulus was detected. As the patient was haemodynamically stable, the procedure was ended. The next day another TOE revealed a shunt volume (left-to-right ventricle) between 50% and 60%. Because the defect was partly located between the stent struts of the ACURATE valve decision was made to fix this leakage with implantation of a further valve and we chose an EVOLUT Pro 29 mm (Medtronic Inc., Minneapolis, MN, USA). The valve-in-valve was implanted 2–3 mm below the lower edge of the first valve, more towards the left ventricular outflow tract (LVOT) with excellent result: VSD was reduced to a very small residual shunt without any hemodynamic relevance. Discussion We suggest that an iatrogenic VSD located near the annulus may be treated percutaneously in a bail-out situation with implantation of a second valve that should be implanted slightly more into the LVOT to cover the VSD.


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