Israel Medical Association Journal

Background: Many countries have adopted a mandatory routine pulse oximetry screening of newborn infants to identify babies with otherwise asymptomatic critical congenital heart disease (CCHD).

Objectives: To describe the current status of pulse oximetry CCHD screening in Israel, with a special emphasis on the experience of the Shaare Zedek Medical Center.

Methods: We review the difficulties of the Israeli Medical system with adopting the SaO₂ screening, and the preliminary results of the screening at the Shaare Zedek Medical Center, both in terms of protocol compliance and CCHD detection.

Results: Large scale protocol cannot be implemented in one day, and regular quality assessment programs must take place in order to improve protocol compliance and identify the reasons for protocol failures.

Conclusions: Quality control reviews should be conducted soon after implementation of the screening to allow for prompt diagnosis and quick resolution

Background: Congenital heart defects (CHD) may be associated with neurodevelopmental abnormalities mainly due to brain hypoperfusion. This defect is attributed to the major cardiac operations these children underwent, but also to hemodynamic instability during fetal life. Advances in imaging techniques have identified changes in brain magnetic resonance imaging (MRI)in children with CHD.

Objectives: To examine the correlation between CHD and brain injury using fetal brain MRI.

Methods: We evaluated 46 fetuses diagnosed with CHD who underwent brain MRI. CHD was classified according to in situs anomalies, 4 chamber view (4CV), outflow tracts, arches, and veins as well as cyanotic or complex CHD. We compared MRI results of different classes of CHD and CHD fetuses to a control group of 113 healthy brain MRI examinations.

Results: No significant differences were found in brain pathologies among different classifications of CHD. The anteroposterior percentile of the vermis was significantly smaller in fetuses with abnormal 4CV. A significantly higher biparietal diameter was found in fetuses with abnormal arches. A significantly smaller transcerebellar diameter was found in fetuses with abnormal veins. Compared to the control group, significant differences were found in overall brain pathology in cortex abnormalities and in extra axial findings in the study group. Significantly higher rates of overall brain pathologies, ventricle pathologies, cortex pathologies, and biometrical parameters were found in the cyanotic group compared to the complex group and to the control group.

Conclusions: Fetuses with CHD demonstrate findings in brain MRI that suggest an in utero pathogenesis of the neurological and cognitive anomalies found during child development.

Cardiac patients of all ages were managed in the past by internists who specialized in cardiology. During the past 50 years, the medical field has witnessed great strides in the management of congenital heart disease, and thus pediatric cardiology has become a subspecialty in many countries. This review article focuses on the advances in fetal cardiac interventions (FCI) since its inception by our group in 1975. Three major modes of FCI have evolved during the past 42 years: pharmacologic, closed FCI, and open FCI. All treatments require a careful approach by the heart team and are reserved for severe fetal cardiac conditions. They call for prenatal intervention in view of the severity and progressive nature of the diseases that are associated with high fetal morbidity and mortality if left untreated. The well-established pharmacologic FCI approach includes several new and effective agents with recommendations often varying between class I and class IIa and IIb. The advances in prenatal echocardiographic imaging and color flow Doppler has given an impetus to the development of the other FCI modes; however, the need for uterine incision and fetal cardiac bypass in the open technique have limited its advance. Long-term outcomes are still unknown and definite conclusions as to the efficacy and safety of FCI need further investigation, including multicenter trials with long-term data. 

Background: Pulmonary arterial hypertension (PAH) is a significant consequence of congenital heart disease (CHD). Its presence and severity is associated with increased morbidity and mortality. 

Objectives: To evaluate the clinical and demographic characteristics of adults with congenital heart diseases (ADCHD) and PAH at a single center. 

Methods: A prospective registry of all patients with PAH was conducted between 2009 and 2015. 

Results: Thirty-two patients were identified. The mean age at the last visit was 44 years (range 19–77 years). The prevalence of PAH among all ADCHD patients was 6% (95% confidence interval 4.3%–8.4%). A much higher prevalence (53%) was found in patients with Down syndrome. Most patients with PAH had moderate or severe disease. Fifteen patients (47%) were treated with pulmonary vasodilators and 6 (19%) with combination therapy. The average World Health Organization functional class was 2.6. Morbidity included cerebral vascular accident or transient ischemic attack in 22% (mostly in patients with right-to-left shunt) and arrhythmia in 37% of the patients. During a median follow-up of 3.5 years, 5 patients (15.6%) died. Of 13 women with no mental retardation, 11 were or had been married and all had children (between 1 and 13, mean 3.3). 

Conclusions: Patients with congenital heart disease and PAH have significant morbidity and mortality. PAH is more prevalent in patients with Down syndrome. While pulmonary pressure during the reproductive years was not always known, 27% of women with PAH at the time of the study were multiparous.

 

Objectives: To assess the role of electrocardiography-gated CT-angiography (ECG-CTA) in the routine evaluation of CHD in neonates and infants particularly for the assessment of extracardiac findings.

Methods: The study cohort comprised 40 consecutive patients who underwent trans-thoracic echocardiography (TTE) and ECG-CTA. TTE and ECG-gated CTA findings regarding extracardiac vascular structures, coronary arteries and airways were compared with surgical or cardiac catheterization findings. Scans were evaluated for image quality using a subjective visual scale (from 1 to 4). Effective radiation dose was calculated for each scan.

Results: Median age was 28 ± 88 days and mean weight 3.7 ± 1.5 kg. Diagnostic quality was good or excellent (visual image score 3–4) in 39 of 40 scans (97.5%). ECG-CTA provided important additional information regarding extracardiac vascular structures and airway anatomy, complementing TTE in 75.6% of scans. Overall sensitivity of ECG-gated CTA for detecting extracardiac findings as compared with operative and cardiac catheterization findings was 97.6%. The calculated mean effective radiation dose was 1.4 ± 0.07 mSv (range 1.014–2.3 mSv).

Conclusions: ECG-CTA is an accurate modality for demonstrating extracardiac structures in complex CHD. It provides important complementary information to TTE regarding extracardiac vascular structures and coronary artery anatomy. This modality may obviate the need for invasive cardiac catheterization, thus exposing the patient to a much lower radiation dose. 

Objectives: To investigate the predictive value of BNP levels regarding the severity of the postoperative course in infants undergoing surgical repair of congenital heart disease.

Methods: We conducted a prospective comparative study. Plasma BNP levels in infants aged 1–12 months with congenital heart disease undergoing complete repair were measured preoperatively and 8, 24 and 48 hours postoperatively. Demographic and clinical data included postoperative inotropic support and lactate level, duration of mechanical ventilation, intensive care unit (ICU) and hospitalization stay.

Results: Cardiac surgery was performed in 19 infants aged 1-12 months. Preoperative BNP level above 170 pg/ml had a positive predictive value of 100% for inotropic score ≥ 7.5 at 24 hours (specificity 100%, sensitivity 57%) and 48 hours (specificity 100%, sensitivity 100%), and was associated with longer ICU stay (P = 0.05) and a trend for longer mechanical ventilation (P = 0.12). Similar findings were found for 8 hours postoperative BNP above 1720 pg/ml. BNP level did not correlate with measured fractional shortening.

Conclusions: In infants undergoing heart surgery, preoperative and 8 hour BNP levels were predictive of inotropic support and longer ICU stay. These findings may have implications for preplanning ICU loads in clinical practice. Further studies with larger samples are needed.

Background: Parental knowledge of their child’s heart disease, while often overlooked, contributes to compliance and reduces anxiety. Prior studies have shown that 36% of parental diagnostic descriptions are incorrect.

Objectives: To assess parental knowledge and attitudes among outpatients at a hospital pediatric cardiology clinic.

Methods: Seventy-four families completed a questionnaire in which they described their child’s condition and stated their attitude towards dental hygiene and future prenatal diagnosis.

Results: Eighteen percent of the parents failed to describe their child’s malformation correctly. We found that parental understanding of the heart defect correlated with parental education. Future prenatal diagnosis was considered by 88% of families, and termination of pregnancy by 40%. Only 40% of children were aware of their heart problem. Children of parents who were ignorant about the condition tended to lack knowl­edge themselves. An additional finding was that 68% of Jewish families turn to non-medical personnel for medical advice - an interesting finding not hitherto addressed.

Conclusions: Ignorance of their child’s problem did not correlate with its severity or complexity but rather with parental background: the less educated the parent, the more likely was the problem perceived incorrectly.