Objectives:

Most researchers use the time domain and spectral analysis in the assessment of heart rate variability (HRV), while others use either the time or frequency domain measures. In this study, we investigated the presence of correlation between the time and frequency domain indices of HRV in normal healthy children and in patients with atrial septal defect (ASD).

Study design:

A total of 60 children, 28 with ASD and 32 healthy children, were recruited. Time domain measures and frequency domain measures were analyzed from the 24-hour Holter ECG records. Correlation between time domain measures and frequency domain measures as well as correlation within the time domain measures was computed in each group.

Results:

There was a positive correlation among all the measurements except the low- (LF) and high- (HF) frequency (LF/HF) ratio which was negatively correlated. The degree of correlation was stronger in some variables and weak in others.

Conclusion:

We have shown that time domain measures are correlated with frequency domain measures in both ASD patients and in healthy children. Some of these indices are so strongly correlated with each other that they can be used interchangeably.

Deletions of the long arm of chromosome 18 have been previously reported in many patients. Most cases involve the more distal regions of the long arm (18q21.1->qter). However, proximal interstitial deletions involving 18q11.2 are extremely rare. Here we report on a 14-month-old female with a 4.7 Mb (19,667,062-24,401,876 hg19) de novo interstitial deletion within chromosomal band 18q11.2, which includes GATA6 and 24 other RefSeq genes. The clinical features of our patient include complex congenital heart defects, a double outlet right ventricle, a subaortic ventricular septal defect, D-malposed great arteries, an atrial septal defect, a dysplastic aortic valve and patent ductus arteriosus. In addition, she had renal anomalies-a duplicated collecting system on the left and mild right hydronephrosis. These heart and renal defects are not reported in other patients with 18q proximal interstitial deletions. Heterozygous point mutations in GATA6, encoding for a zinc finger transcription factor, have been shown to cause congenital heart defects. Given the well-established biological role of GATA6 in cardiac development, a deletion of GATA6 is very likely responsible for our patient's complex congenital heart defects. This is the smallest and most proximal 18q11.2 deletion involving GATA6 that is associated with complex congenital heart disease and renal anomalies. © 2013 Wiley Periodicals, Inc.

Isolated single coronary artery in the presence of an atrial septal defect (ASD) is a rare congenital anomaly. There are no clear guidelines regarding the management of ASD in these patients. Transcatheter closure of the ASD in the presence of a single coronary artery that has a retro-aortic course is a matter of concern because of the possible risk of coronary impingement by the left atrial disc. Here we report the first case of an ASD device closure in a patient with a retro-aortic right coronary artery. The immediate results were good; however, long-term follow up is warranted to look for late complications.

Concentrations of B-type natriuretic peptide (BNP) are recognised as a reliable marker of ventricular dysfunction in adults. In this study, plasma levels of BNP were determined in children with congenital heart disease (CHD) involving a left-to-right shunt, and were correlated with the shunt volume.Seventy-six children (38 boys/38 girls, mean age 22.4 months) with CHD (Group A: 31 with atrial septal defect [ASD], 23 with ventricular septal defect [VSD], 8 with ASD and VSD, 14 with patent ductus arteriosus [PDA]) and 34 healthy children (group B) were studied. BNP was measured by chemiluminescent microparticle immunoassay in all children. The amount of shunt (the ratio of pulmonary blood flow/systemic blood flow: Qp/Qs) was measured using Doppler velocimetry and two-dimensional echocardiography. A haemodynamically significant left-to-right shunt was defined as Qp/Qs>1.5. Correlations were evaluated between all patient groups and healthy subjects and BNP was compared with echocardiographic data reflecting right and left ventricle volume overload.Thirty-four children of group A had Qp/Qs>1.5 (group A1) and 42 Qp/Qs<1.5 (group A2). BNP levels were higher in group A1 than group A2 (p=0.015), while there were no significant differences in BNP between group A2 and group B (p=0.79). BNP 24.4 pg/ml was determined as the cut-off point to identify patients with Qp/Qs>1.5. BNP values were similar among patients with ASD and VSD, but they were significantly higher in patients with PDA. BNP was positively correlated with Qp/Qs (r=0.59, p<0.001), and with the pulmonary artery velocity (r=0.27) and gradient (r=0.49), while there was a negative correlation with ejection fraction (r=-0.14). BNP levels were significantly higher in 10 infants with clinical signs of heart failure (p=0.025).These results, which are consistent with previous reports, suggest a possible role of BNP as an early diagnostic marker of the significance of shunt in children with CHD.

BACKGROUND:

Structural heart interventions require the use of relatively large-diameter delivery sheaths or latex nylon net balloon catheters, which results in a relatively large-diameter venous puncture point. At present, femoral venous hemostasis is achieved by manual compression. A temporary figure-of-eight subcutaneous suture has been introduced to achieve immediate postprocedural femoral venous hemostasis after using a ≥24Fr sheath in an adult. This method is not well evaluated in children.

METHOD:

We report our experience using this technique in children treated with 7-14Fr sheaths. From May 2009 to March 2012, 104 children (ages ranging from 3 to 10 years, mean 8.1 ± 1.6 years) with atrial septal defects (n = 86) and pulmonary valve stenosis (n = 18) underwent percutaneous transcatheter interventions. Sheath sizes used were ≥7Fr (7Fr, n = 5; 8Fr, n = 29; 9Fr, n = 14; 10Fr, n = 15; 12Fr, n = 19; 14Fr, n = 22).

RESULT:

A total of 102 patients had immediate femoral vein hemostasis, and 2 developed a femoral vein hematoma requiring manual compression. One patient was diagnosed with a femoral artery pseudoaneurysm during hospitalization. On follow-up, there was no evidence of hematoma or thrombosis.

CONCLUSION:

The "figure-of-eight" suture technique is effective and safe, achieving immediate hemostasis after the use of large femoral vein sheaths in children.

BACKGROUND:

There is a scarcity of data comparing percutaneous and surgical closure of the secundum atrial septal defect (ASD).

OBJECTIVES:

Assessment of safety and efficacy of both methods of treatment in a referral center affiliated with the Ministry of Health.

METHODS:

Observational, prospective, non-randomized study of two cohorts of children and adolescents younger than 14 years, treated by catheterization or surgery. Data was collected prospectively in the percutaneous group (A) and retrospectively in the surgical group (B).

RESULTS:

A total of 75 patients (pts) were enrolled in group A from April 2009 to October 2011 and 105 pts were treated in group B from January 2006 to January 2011. Age was older and weight was higher in group B and the ASD diameter was similar in both groups. Technical success was achieved in all procedures and there were no deaths. Complications (most minor) occurred in 68% of group B and 4% of A (p < 0.001). Rates of total occlusion or non-significant residual shunts were similar in both groups. Median hospitalization time was 1.2 days in group A and 8.4 days in group B (p < 0.001).

CONCLUSION:

Both treatment modalities are safe and effective, showing excellent outcomes. However, the percutaneous treatment has lower morbidity and shorter in-hospital stay length. These observations support the concept that percutaneous treatment of atrial septal defects should be regarded as the method of choice to manage selected patients with this condition.

Levoatrial cardinal vein is a rare congenital anomaly of the systemic veins. It is commonly associated with left-sided obstructive conditions such as aortic atresia, mitral atresia, and cor triatriatum. We report the case of a 14-year-old boy who was undergoing surgery for correction of a secundum atrial septal defect. Intraoperatively, we discovered that he had a levoatrial cardinal vein and no brachiocephalic vein. However, collateral vessels provided adequate flow to the right atrium, and the patient's left-sided venous pressure was not excessive, so we ligated the levoatrial cardinal vein and directly repaired the septal defect. Postoperatively, the left venous drainage was satisfactory and the patient was asymptomatic. In addition to our patient's case, we discuss the embryology, diagnosis, and treatment of levoatrial cardinal vein.

aim: Atrial septal defect (ASD) is one of the most common causes of congenital heart disease manifested in adulthood.

Aim:

To describe clinical and likelihood picture of adults over 60 years born with an ASD type II. methods: We performed a retrospective study of adult'sASD referred toourinstitutionfrom 1985 through 2010. Clinical, electrocardiographic, echocardiographic and hemodynamic data were reviewed. On follow up study, patients were investigated by echocardiography, ECG and assessed for quality of life by a questionnaire results: Among forty ASD type II manifested in adulthood which were referred to our department of cardiology five cases of ASD manifested in the sixth decades (2 men and 3 women). Complaints were dyspnea and palpitations in 4 cases and chest pain in only one patient. Slight anterior chest deformity was present in the older patient. Systolic murmur was found in the 3rd left intercostals space and the pulmonary second heart sound was accentuated in all patients. Complete right bundle branch block and right ventricular hypertrophy were found in all cases. Three patients presented atrial fibrillation. There was marked cardiomegaly in four patients. The pulmonary arteries were markedly enlarged and the peripheral vascular markings were increased. Echocardiographic data revealed large secundum ASD (mean 20 mm, ranged between 10 and 30mm), severe systolic pulmonary pressure in two cases (>5O mmhg). MeanQP/QS was 2.2 and contrast revealed bidirectional shunt in one patient. All patients were studied by venous cardiac catheterization. They showed a significant increase in the oxygen content of right atrial blood. Three patients underwent surgical atrial septal defect closure under general anesthesia. There were no operative or peri operative deaths. At mean follow up of 50±75 months, there was one late death from heart failure in a patient with advanced preoperative heart failure. The oldest patient is in the medical group and he is 75years old. Most survival patients remain in good clinical condition. Some of them were symptomatic at the last follow up and complained of shortness of breath on effort and palpitations in two cases. Two patients were in chronic atrial fibrillation developed during follow up. However, chest RX showed reduction in cardiothoracic ratio postoperatively. Echocardiographic examination confirmed that there was no residual shunt in across the atrial septum in any patient. Systolic pulmonary pressure felled only in 2 patients in the surgery group.

Conclusion:

To our knowledge, thesepatients havealongue life span, although survivors with ASD described in the world. There is a lack of evidence regarding treatment options for adults with an ASD aged more than 60years. Given the higher risks of surgery in advanced age, the defect should be repaired as early as possible to prevent hemodynamic complications.

Complex ostium secundum type atrial septal defect (ASD) is a definition used for large (stretched diameter over 26 mm with deficient rim) or multiple ASDs or multi-fenestrated septum or ASDs with redundant and aneurysmal septal rims. Compared to simple defects, transcatheter ASD closure with Amplatzer septal occluder (ASO) is relatively challenging in these cases, and different techniques have been defined to increase procedure success. We report three adult patients with complex ostium secundum type ASDs that were closed with ASO device using three different techniques. The first case was a 36-year-old female patient with complaints of dyspnea, palpitation and fatigue. A complex ostium secundum type ASD was diagnosed, and the defect was closed with a 36 mm ASO device using a left upper pulmonary vein technique. The second case was a 47-year-old female patient with complaints of dyspnea and palpitation. A large and complex ASD was detected, and the defect was closed with a 28 mm device using a left atrial roof technique supported by non-inflated balloon. The third case was a 40-year-old female patient who presented with complaints of dyspnea and palpitation. Complex ASD was diagnosed, and the defect was closed with a 32 mm device using a partially inflated balloon technique. In large and complex ASDs, the classical implantation technique of an ASO device may fail at any time. The knowledge and application of different techniques that orient the left atrial disc parallel to the septum may increase the procedure success and decrease complications.