scholarly journals Left Ventricular Noncompaction and Congenital Heart Disease Increases the Risk of Congestive Heart Failure

2020 ◽  
Vol 9 (3) ◽  
pp. 785 ◽  
Author(s):  
Keiichi Hirono ◽  
Yukiko Hata ◽  
Nariaki Miyao ◽  
Mako Okabe ◽  
Shinya Takarada ◽  
...  

Background: Left ventricular noncompaction (LVNC) is a hereditary cardiomyopathy that is associated with high morbidity and mortality rates. Recently, LVNC was classified into several phenotypes including congenital heart disease (CHD). However, although LVNC and CHD are frequently observed, the role and clinical significance of genetics in these cardiomyopathies has not been fully evaluated. Therefore, we aimed to evaluate the impact on the perioperative outcomes of children with concomitant LVNC and CHD using next-generation sequencing (NGS). Methods: From May 2000 to August 2018, 53 Japanese probands with LVNC (25 males and 28 females) were enrolled and we screened 182 cardiomyopathy-associated genes in these patients using NGS. Results: The age at diagnosis of the enrolled patients ranged from 0 to 14 years (median: 0.3 months). A total of 23 patients (43.4%) were diagnosed with heart failure, 14 with heart murmur (26.4%), and 6 with cyanosis (11.3%). During the observation period, 31 patients (58.5%) experienced heart failure and 13 (24.5%) developed arrhythmias such as ventricular tachycardia, supraventricular tachycardia, and atrioventricular block. Moreover, 29 patients (54.7%) had ventricular septal defects (VSDs), 17 (32.1%) had atrial septal defects, 10 had patent ductus arteriosus (PDA), and 7 (13.2%) had Ebstein’s anomaly and double outlet right ventricle. Among the included patients, 30 underwent surgery, 19 underwent biventricular repair, and 2 underwent pulmonary artery banding, bilateral pulmonary artery banding, and PDA ligation. Overall, 30 genetic variants were identified in 28 patients with LVNC and CHD. Eight variants were detected in MYH7 and two in TPM1. Echocardiography showed lower ejection fractions and more thickened trabeculations in the left ventricle in patients with LVNC and CHD than in age-matched patients with VSDs. During follow-up, 4 patients died and the condition of 8 worsened postoperatively. The multivariable proportional hazards model showed that heart failure, LV ejection fraction of < 24%, LV end-diastolic diameter z-score of > 8.56, and noncompacted-to-compacted ratio of the left ventricular apex of > 8.33 at the last visit were risk factors for survival. Conclusions: LVNC and CHD are frequently associated with genetic abnormalities. Knowledge of the association between CHD and LVNC is important for the awareness of clinical implications during the preoperative and postoperative periods to identify the populations who are at an increased risk of additional morbidity.

2005 ◽  
Vol 79 (4) ◽  
pp. 1463-1464
Author(s):  
Hiroo Takayama ◽  
Masahide Chikada ◽  
Shinichi Takamoto ◽  
Akihiko Sekiguchi ◽  
Akira Ishizawa

2017 ◽  
Vol 9 (3) ◽  
pp. 364-367
Author(s):  
Abid Iqbal ◽  
Sabarinath Menon ◽  
Baiju S. Dharan ◽  
Kapilamoorthy Tirur Raman ◽  
Jayakumar Karunakaran

Submitral aneurysms are rare clinical entities occurring predominantly in young adults of African descent. A host of etiologies have been proposed for this entity. We present a unique case of submitral aneurysm which developed after pulmonary artery banding in a three-year-old girl with complex congenital heart disease. The aneurysmal sac was burrowing into the interatrial septum.


2021 ◽  
Vol 12 (2) ◽  
pp. 213-219
Author(s):  
R. Allen Ligon ◽  
Larry A. Latson ◽  
Mark M. Ruzmetov ◽  
Kak-Chen Chan ◽  
Immanuel I. Turner ◽  
...  

Background: Surgical pulmonary artery banding (PAB) has been limited in practice because of later requirement for surgical removal or adjustment. The aim of this study is to describe our experience creating a dilatable PAB via transcatheter balloon dilation (TCBD) in congenital heart disease (CHD) patients. Methods: Retrospective chart review of adjustable PAB—outline anatomical variants palliated and patient outcomes. Results: Sixteen patients underwent dilatable PAB—median age 52 days (range 4-215) and weight 3.12 kg (1.65-5.8). Seven (44%) of the patients were premature, 11 (69%) had ventricular septal defect(s) with pulmonary over-circulation, four (25%) atrioventricular septal defects, and four (25%) single ventricle physiology. Subsequent to the index procedure: five patients have undergone intracardiac complete repair, six patients remain well palliated with no additional intervention, and four single ventricles await their next palliation. One patient died from necrotizing enterocolitis (unrelated to PAB) and one patient required a pericardiocentesis postoperatively. Five patients underwent TCBD of the PAB without complication—Two had one TCBD, two had two TCBD, and another had three TCBD. The median change in saturation was 14% (complete range 6-22) and PAB diameter 1.7 mm (complete range 1.1-5.2). Median time from PAB to most recent outpatient follow-up was 868 days (interquartile range 190-1,079). Conclusions: Our institution has standardized a PAB technique that allows for transcatheter incremental increases in pulmonary blood flow over time. This methodology has proven safe and effective enough to supplant other institutional techniques of limiting pulmonary blood flow in most patients—allowing for interval growth or even serving as the definitive palliation.


Thorax ◽  
1968 ◽  
Vol 23 (4) ◽  
pp. 385-391 ◽  
Author(s):  
J. M. Reid ◽  
R. S. Barclay ◽  
E. N. Coleman ◽  
J. G. Stevenson ◽  
T. M. Welsh ◽  
...  

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