BACKGROUNDRight dominant heart (RDH) in fetuses can occur with a number of cardiac as well as noncardiac anomalies. Analysis of the enlargement of the right cardiac chamber in the fetus remains a major challenge for sonographers and echocardiographers. The aim of this study was to report the experience with prenatal diagnosis of RDH in the fetuses over a 7-year period.

METHODSFetuses with prenatal diagnosis of RDH from July 2009 to July 2016 were evaluated in two different categories: according to the gestational age, Group I (n = 154, second trimester) and Group II (n = 298, third trimester); and according to the fetal echocardiography diagnosis, Group A (n = 452, abnormal cardiac structure) and Group B (n = 90, normal cardiac structure). Differences in categorical variables were assessed by Chi-square exact test and continuous variables were evaluated by independent Student's t-test or Mann-Whitney U-test depending on parametric or nonparametric nature of the data.

RESULTSOver a 7-year period, 452 fetuses were referred for the assessment of suspected RDH. Left-sided obstructive lesions were observed most frequently in the fetuses with RDH. When comparing Group I with Group II and Group A with Group B, the latter groups exhibited significant differences in the right/left ventricle (RV/LV) ratio (1.435 vs. 1.236, P = 0.002; 1.309 vs. 1.168, P = 0.047), RV width Z-score (1.626 vs. 1.104, P < 0.001; 1.553 vs. 0.814, P = 0.014), and above +2 cutoff percentages (14.3% vs. 22.5%; P = 0.038; 21.5% vs. 12.2%, P = 0.046). Multivariable logistic regression revealed no variables associated with perinatal survival.

CONCLUSIONSThe study demonstrates that RDH warrants careful attention to the possible presence of a structural cardiac anomaly, especially left-sided obstructive lesions. A diagnosis of RDH is best supported by a combination of the RV Z-score and RV/LV ratio. Most of the fetuses with RDH and structurally normal hearts had favorable outcomes.

Echocardiography ; Female ; Fetal Diseases ; diagnosis ; Fetal Heart ; abnormalities ; Heart Ventricles ; abnormalities ; Humans ; Pregnancy ; Prenatal Diagnosis ; methods ; Ultrasonography, Prenatal

The management of pulmonary atresia with intact ventricular septum (PA/IVS) remains controversial. The goal of separating systematic and pulmonary circulation can be achieved by biventricular or uni-ventricular (Fontan or one and a half ventricle repair) strategies. Although outcomes have been improved, these surgical procedures are still associated with high mortality and morbidity. An optimal strategy for definitive repair has yet to be defined. We searched databases for genetically randomized controlled trials (RCTs) comparing biventricular with uni-ventricular repair for patient with PA/IVS. Data extraction and quality assessment were performed following the guidelines of the Cochrane Collaboration. Primary outcome measures were overall survival, and secondary criteria included exercise function, arrhythmia-free survival and treatment-related mortality. A total number of 669 primary citations were screened for relevant studies. Detailed analysis revealed that no RCTs were found to adequately address the research question and no systematic meta-analysis would have been carried out. Nevertheless, several retrospective analyses and case series addressed the question of finding right balance between biventricular and uni-ventricular repair for patient with PA/IVS. In this review, we will discuss the currently available data.
Arrhythmias, Cardiac ; physiopathology ; prevention & control ; Cardiac Valve Annuloplasty ; methods ; mortality ; Fontan Procedure ; methods ; mortality ; Heart Defects, Congenital ; mortality ; pathology ; surgery ; Heart Ventricles ; abnormalities ; pathology ; surgery ; Humans ; Pulmonary Atresia ; mortality ; pathology ; surgery ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

This study was aimed to establish an experimental mouse model of combined transgenic inhibition of both multifunctional Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) and inward rectifier potassium current (Ik1), and to observe whether the specific inhibition of both CaMKII and Ik1 can bring about any effects on cardiac remodeling. Mice were divided into 4 groups: wild type (WT), CaMKII inhibited (AC3-I), Ik1 inhibited (Kir2.1-AAA) and combined inhibition of both CaMKII and Ik1 (AC3-I+Kir2.1-AAA). Mice in each group received electrocardiogram (ECG) and echocardiography examination. ECG in the condition of isoproterenol (ISO) injection was also checked. The whole cell patch clamp technique was used to measure Ik1 and the transient outward potassium current (Ito) from enzymatically isolated myocytes of left ventricle. In the condition of basal status, no significant changes of heart rate, PR interval and QRS interval were observed. No mouse showed ventricular arrhythmias in all of the 4 groups. After ISO injection, each group presented no significant ventricular arrhythmias either. The indexes measured by M-mode (motion-mode) and two-dimensional echocardiography had no significant differences among the four groups. Ik1 in AC3-I group was significantly higher than those in other three groups (P < 0.01) because of the results brought about by CaMKII inhibition. Among the latter three groups, both Kir2.1-AAA group and AC3-I+Kir2.1-AAA group had a significant reduced Ik1 compared with that of WT group, which was due to the Ik1 inhibition (P < 0.01). Ito in AC3-I group was higher than that of the other three groups (P < 0.01), but there were no significant differences in Ito among WT, Kir2.1-AAA and AC3-I+Kir2.1-AAA groups. Thus, combined transgenic myocardial CaMKII and Ik1 inhibition eliminated the up-regulation of Ik1 in CaMKII inhibited mice, and had no effects on cardiac remodeling including heart structure and function as well as arrhythmias at the basic and ISO conditions. The results of this study may provide a basis for the further investigation of combined inhibition of CaMKII and Ik1 in pathogenic cardiac remodeling.
Animals ; Arrhythmias, Cardiac ; Brugada Syndrome ; Calcium-Calmodulin-Dependent Protein Kinase Type 2 ; physiology ; Cardiac Conduction System Disease ; Disease Models, Animal ; Electrocardiography ; Heart ; physiology ; Heart Conduction System ; abnormalities ; Heart Ventricles ; Isoproterenol ; Mice ; Mice, Transgenic ; Patch-Clamp Techniques ; Potassium Channels, Inwardly Rectifying ; physiology ; Up-Regulation ; Ventricular Remodeling

OBJECTIVETo evaluate the value of echocardiography in the diagnosis of noncompaction of the ventricular myocardium (NCVM) and to elucidate the echocardiographic characteristics of NCVM.

METHODSThis study included 53 patients (28 boys and 25 girls), with an age for initial diagnosis of 15 days to 18 years, who were diagnosed with NCVM by echocardiography between May 2006 and May 2015. Transthoracic two-dimensional echocardiography and color Doppler were performed for qualitative diagnosis, and the end-diastolic non-compacted layer/compacted layer (N/C) ratio measured in the parasternal ventricular short-axis sectional view was selected as the criterion for quantitative diagnosis.

RESULTSThe excessively prominent ventricular trabeculae and deep inter-trabecular recesses were all seen in 53 cases, and the blood flow in the cardiac chambers was connected to the inter-trabecular recesses. The areas involved in NCVM were mainly the apex (100%) and the middle segment of the left ventricular lateral wall (98%), followed by the middle segment of the left ventricular posterior wall (49%) and the middle segment of the left ventricular inferior wall (42%). In 53 children with NCVM, the N/C ratio was 4.3±1.9 (2.1-10.0). Cardiac insufficiency was found in 83% (44/53) of the children with NCVM, and the left ventricular ejection fraction for these children was (43±9)%.

CONCLUSIONSEchocardiography can be used in the qualitative and quantitative diagnosis of NCVM and in the evaluation of cardiac function. The apex and the middle segment of the left ventricular lateral wall are often involved in NCVM, accompanied by decrease in the left ventricular ejection fraction.

Adolescent ; Child ; Child, Preschool ; Echocardiography ; Female ; Heart Ventricles ; abnormalities ; diagnostic imaging ; Humans ; Infant ; Infant, Newborn ; Male ; Stroke Volume ; Ventricular Function, Left

OBJECTIVETo discuss the diagnosis and surgical treatment of congenital vascular ring and prognostic factors.

METHODSThe clinic data of 42 cases of congenital vascular from January 2010 to December 2013 was analyzed retrospectively (accounting for 1.04% congenital heart operations over the same period ). There were 26 male and 16 female patients, aged at surgery 24 days to 6 years (average 10.7 months). The diagnosis including pulmonary artery sling in 26 cases, double aortic arch in 10 cases, right aortic arch with aberrant left subclavian artery in 3 cases, pulmonary artery sling and right aortic arch with vagus left subclavian artery in 2 cases, pulmonary artery sling and left aortic arch with vagus right subclavian artery in 1 case. In addition to 4 cases the remaining 38 patients were still combined with other cardiovascular malformations. Thirty-six cases of children underwent spiral CT airway remodeling, 23 children underwent fiber- bronchoscopy. In addition to 2 cases of airway abnormalities not seen, the rest of the children were present in varying degrees in different parts of tracheal stenosis or tracheomalacia. All patients underwent surgical correction of congenital vascular ring, concomitant heart deformity correction surgery according to the situation (7 cases of atrial septal defect repair, 3 of ventricular septal defect repair, 1 of mitral valvuloplasty, 1 of bi-Glenn, 1 of coarctation of the aorta correction with ventricular septal defect repair, 1 of trilogy of Fallot correction). One case suffered resection of tracheal stenosis and Slide tracheoplasty simultaneously, another case was implanted tracheal stenting postoperatively after pulmonary artery sling correction.

RESULTSThree patients died in hospital (7.1%), the cause of death were recurrent granuloma formation postoperatively. Remaining 39 patients were successfully discharged. The median time of overall survival of children with mechanical ventilation was 14 h (22 h) (M (Q(R))), median ICU residence time was 5 d (8 d), and the median in-hospital time was 19 d (9 d). Tracheal intubation time and postoperative ICU duration time in children with pulmonary artery sling children were much more than in children with double aortic arch (23 h (123 h) vs. 9 h(9 h), 7 d (13 d) vs. 4 d (2 d)), but the difference were not statistically significant. There was significant difference in the duration of hospitalization between the patient with the pulmonary artery sling and double aortic arch (23 d (9 d) vs. 16 d(6 d)) (χ(2) = 10.157, P = 0.006).

CONCLUSIONSThe recent results of surgical treatment of congenital vascular ring is safe and effective. The extent and scope of tracheal stenosis and tracheomalacia is a critical influence prognosis.

Aorta ; abnormalities ; Aorta, Thoracic ; abnormalities ; Child ; Child, Preschool ; Female ; Heart Defects, Congenital ; surgery ; Heart Septal Defects, Ventricular ; surgery ; Heart Ventricles ; abnormalities ; Humans ; Infant ; Infant, Newborn ; Male ; Prognosis ; Pulmonary Artery ; abnormalities ; Retrospective Studies ; Tomography, Spiral Computed ; Trachea ; surgery ; Tracheal Stenosis ; surgery ; Treatment Outcome

OBJECTIVETo evaluate the efficacy and experience in right ventricular pacing-percutaneous balloon aortic valvuloplasty (RVP-PBAV) for congenital aortic stenosis (AS).

METHODA total of sixteen children with AS accepted the treatment with RRVP-PBAV. The patients were at ages 6 months to 15 years, their median age was 5.4 years. Their body weight was between 8.5 and 59.0 kg, average (22.3 ± 16.5) kg. The gradient pressure across the aortic valve was measured for all the patients and aortic regurgitation was observed. The follow-up time ranged from 1 month to 5.5 years.

RESULTAll patients underwent RVP-PBAV successfully. The ratios of balloon/valve were 0.86 to 1.12. The gradient pressure varied from preoperative Δp = (96 ± 32) mmHg (1 mmHg = 0.133 kPa) to the immediate postoperative ΔP = (41 ± 26) mmHg, (P < 0.05). One case had postoperative restenosis, and 3 cases were complicated with bicuspid aortic valve deformity.

CONCLUSIONThe treatment with RVP-PBAV for congenital aortic stenosis is safe and reliable. Rapid ventricular pacing is a safe procedure to stabilize the balloon during balloon aortic valvuloplasty and may decrease the incidence of aortic insufficiency.

Adolescent ; Aorta ; Aortic Valve ; abnormalities ; Aortic Valve Insufficiency ; Aortic Valve Stenosis ; therapy ; Balloon Valvuloplasty ; methods ; Body Weight ; Cardiac Surgical Procedures ; Child ; Child, Preschool ; Follow-Up Studies ; Heart Defects, Congenital ; Heart Valve Diseases ; Heart Ventricles ; Humans ; Infant ; Postoperative Period ; Treatment Outcome ; Vascular Malformations

Aorta ; abnormalities ; Child ; Heart Defects, Congenital ; diagnosis ; Heart Ventricles ; abnormalities ; Humans ; Male

This paper was aimed to explore the features of single ventricle with dual source computed tomography (DSCT) and the value of the modality in diagnosis of the single ventricle disease. Dual-source computed tomography imaging of 14 cases of single ventricle compared with the results of surgery and cardiac catheter were retrospectively analyzed. Firstly, 14 cases were classified into three types according to Anderson classification method, including 7 cases(50. 0%) left ventricular type, 3 cases(21. 4%) right ventricular type and 4 cases (28. 6%) undecided ventricular type. Secondly, in accompanying malformation respect, pulmonary stenosis, bilateral superior vena cava, atrial septal defect (ASD) and common atrioventricular valve were presented frequently. Thirdly, affiliated cardiac chamber of left ventricular type were mostly in front of the major cardiac chamber (5 cases,71. 4%), while affiliated cardiac chamber of right ventricular type were behind of the major cardiac chamber totally. Fourthly, more than half of aortas and pulmonary arteries stemming from common cardiac chamber can be seen (8 cases, 57. 1%). Meanwhile the aorta valves were more on the right of pulmonary valve (9 cases, 64. 3%). The classification of 10 cases of single ventricle was compared with the results of surgery, and the coincidence rate is 100%. DSCT can diagnose the single ventricle disease accurately. The aorta, pulmonary artery and coronary artery can be displayed completely and simultaneously as well.
Adolescent ; Child ; Child, Preschool ; Female ; Heart Ventricles ; abnormalities ; diagnostic imaging ; Humans ; Infant ; Male ; Radiography, Dual-Energy Scanned Projection ; methods ; Retrospective Studies ; Tomography, X-Ray Computed ; methods

Arterio-Arterial Fistula ; therapy ; Cardiac Catheterization ; Coronary Vessels ; Female ; Heart Septal Defects, Ventricular ; therapy ; Heart Ventricles ; abnormalities ; Humans

We found a case of hypoplasia of vertebral artery with fibromuscular dysplasia in an 82-yr-old Korean female cadaver during a routine dissection course. In the present case, intracranial hypoplasia in left vertebral artery and bilateral origin of posterior inferior cerebellar artery at the vertebrobasilar junction were recognized. Histopathologically, left vertebral artery showed intimal type of fibromuscular dysplasia both in its extracranial and intracranial courses. These results indicate that the association of fibromuscular dysplasia and hypoplasia does exist in the vertebral artery, although the etiologies are not verified yet.
Aged, 80 and over ; Female ; Fibromuscular Dysplasia/*pathology ; Heart Ventricles/abnormalities/*pathology ; Humans ; Republic of Korea ; Vertebral Artery/*pathology