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A patient was delivered at 26 weeks (about 6 months) gestation via an emergency caesarian section. A patent ductus arteriosus (PDA) and atrial septal defect (ASD) were discovered during an echocardiogram 13 days after birth. The patient had catheter-based closure of the PDA and ASD. During a routine echocardiogram to check device placements, it was discovered that there was dilation of the superior vena cava (SVC), and it was suspected that a thrombus was present. Computed tomography angiography (CTA) was completed to better define SVC anatomy and flow acceleration. The CTA demonstrated that there was a double innominate vein., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

The use of medications plays a pivotal role in the management of children with heart diseases. Most children with increased pulmonary blood flow require chronic use of anticongestive heart failure medications until more definitive interventional or surgical procedures are performed. The use of such medications, particularly inotropic agents and diuretics, is even more amplified during the postoperative period. Currently, children are undergoing surgical intervention at an ever younger age with excellent results aided by advanced anesthetic and postoperative care. The most significant of these advanced measures includes invasive and noninvasive monitoring as well as a wide array of pharmacologic agents. This review update provides a medication guide for medical practitioners involved in care of children with heart diseases.

Myocardial performance index (MPI), or Tei index, has become a commonly used parameter for the noninvasive, Doppler-derived assessment of global systolic and diastolic performance of the heart in both adults and children. Normal values have been established in adults and children; however, limited data exist in fetal hearts. The aim of this study was to further elucidate normal values of fetal left (LV) and right ventricle (RV) MPI values in second- and third-trimester fetuses and compare these values with other previously published data. This was a retrospective study to measure MPI in healthy fetuses. After Institutional Review Board approval, 2000 fetal echocardiography studies (FES) were acquired during a period of 4 years. Demographic parameters examined included gestational age (GA), maternal age (MA), and indication for fetal echocardiography. Fetuses with congenital heart disease, arrhythmias, or significant noncardiac fetal anomalies were excluded. The following echocardiography parameters were collected: LV ejection time (LVET), mitral valve close-to-open time (MVCO), RVET, tricuspid valve CO (TVCO), and fetal heart rate. For simplicity, LV and RV MPI values were calculated as follows: LV MPI = MVCO - LVET/LVET and RV MPI = TVCO - RVET/RVET. Four hundred twenty FES met the study criteria. LV MPI was evaluated in 230 and 190 FES in the second and third trimester, respectively. Of the 420 FES, 250 (150 in the second trimester and 100 in the third trimester) had all of the measurements required for RV MPI calculation. MA ranged between 16 and 49 years. Indications for FES included diabetes mellitus (N = 140; 33 %), suspected fetal anomalies on routine obstetrical ultrasound (N = 80; 20 %), autoimmune disorder (N = 60; 14 %), family history of CHD (N = 76; 18 %), medication exposure (N = 22; 5 %), increase nuchal thickness (N = 13; 3 %), and other indications (N = 29; 6 %). Averaged LV and RV MPI values were 0.464 ± 0.08 and 0.466 ± 0.09, respectively. Further analysis based on gestational period showed slightly greater LV and RV MPI values during the third compared with the second trimester, i.e., 0.48 and 0.49, respectively, with no statistically significant difference. There was no significant association of LV and RV MPI with heart rate. To our knowledge, this is the first study to establish normal values of fetal MPI based on a large fetal population from a single tertiary center. LV and RV MPI values were independent of GA and fetal heart rate. MPI is a useful parameter for the assessment of global cardiac function in the fetus and demonstrates good reproducibility with narrow interobserver and intraobserver variability. Its usefulness should be studied in fetal hearts with complex congenital anomalies.

Fenestrated atrial septal defects (F-ASDs) may pose a challenge to device closure; recently, a cribriform device with a minimal connecting intrawaist diameter and large, equal left- and right-sided discs has been designed to cover more than one adjacent defect. This study demonstrates the feasibility and technical aspects of closing F-ASDs using this new device. Sixteen patients between August 2003 and January 2006 were included in this study. The inclusion criterion was the presence of a F-ASD diagnosed by transesophageal echocardiography. One of the three available cribriform ASD device sizes (18, 25, or 35 mm) was implanted. Patients were followed for at least 1 year after the procedure. Thirteen patients had successful cribriform ASD device implantation (median age and weight, 12.5 years and 36 kg, respectively). Ten patients (62%) had an associated atrial septal aneurysm. The mean procedure time was 75.6 +/- 28.5 min and the mean fluoroscopy time 14.8 +/- 6.3 min. The RVEDD was significantly reduced, from a mean of 24.2 mm to 21.0 (p < 0.05). One patient developed atrial tachycardia requiring cardioversion during the procedure. There were no embolic events, heart block, or mortality. Complete closure was 10 of 13 (77 %) the next day and 12 of 13 (92%) at 6 and 12 months. We conclude that the cribriform Amplatzer device can be successfully and safely used in patients with F-ASDs. Complete closure may take up to 6 months.

Background: A variety of transcatheter atrial septal defect (ASD) occluders are currently in use, the most commonly used device is the Amplatzer Septal Occluder (ASO) yet there is no perfect device. The Helex Septal Occluder is a new device (by W.L. Gore & Associates, Inc.) designed to improve the results of transcatheter ASD closure. We report our first experience in closing secundum ASDs with this new device after its recent modifications., Methods: Thirteen patients were selected for Helex device closure with median age of 8 years (2.5-44 years) and median weight 30 Kg (12-96 Kg). Inclusion criteria were: small to moderate Secundum ASDs with sufficient rims by transthoracic echo (TTE) and confirmed by transoesophageal echo (TEE). Two cardiologists carried out the decision of device size. One of the five available Helex ASD device sizes was used (15-35 mm). Follow up TTE was performed the next day, one month, six months and one year later., Results: Twelve patients had successful Helex septal occluder implantation. One patient was switched to Cribriform ASD device during the procedure because of failure of the locking mechanism. Two patients had trivial residual left to right shunt which disappeared at one month follow up in one patient. No embolic event, AV block or mortality was observed., Conclusion: This feasibility study of the novel Helex septal occluder after its recent modification showed that it can successfully and safely close well selected secundum ASDs. There are several advantages over the currently available devices.

Atrial standstill is a rare form of bradyarrhythmia and consists of a transitory or permanent loss of the electrical and mechanical activity of the atria. It has been described in a few cases of long-standing valvular disease, amyloidosis, myocarditis, and muscular dystrophy. We report on a young female with noncompaction cardiomyopathy that progressed to congestive heart failure. Electrocardiogram showed persistence atrial standstill that was confirmed clinically and by electrocardiomyopathy, Doppler two-dimensional echocardiogram, and tissue Doppler imaging. We assume that worsening of asymptomatic noncompaction and progression to the congestive form of cardiomyopathy could be presaged by the presence of persistent atrial standstill. Thus, persistence of atrial standstill in asymptomatic cardiomyopathy is a sign of poor prognosis.

Cardiogenic unilateral pulmonary edema (UPE) is a rare clinical condition and not readily recognized early and managed accordingly. Acute rheumatic fever, which is a common disease in developing countries, does not commonly present with UPE. We report a 13-year-old girl presenting with UPE following acute rheumatic fever mimicking pneumonia. We conclude that UPE should be considered in the differential diagnosis for the patient with clinical criteria of rheumatic fever who presents with unilateral lung opacification. With early recognition and antifailure treatment, it is possible to reduce morbidity and mortality in such patients.