Israel Medical Association Journal

Fetal complete atrioventricular block (CAVB) is usually autoimmune mediated. The risk of developing CAVB is 2% to 3% in anti-Ro/SS-A seropositive pregnancies and it increases 10 times after previous CAVB in siblings. Despite being a rare complication, CAVB carries a 20% mortality rate and substantial morbidity, as about 65% of newborns will eventually need life-long pacing. Once found, fetal CAVB is almost always irreversible, despite aggressive immunotherapy. This poor outcome prompted some research groups to address this situation. All groups followed anti-Ro/SS-A seropositive pregnancies on a weekly basis during the second trimester of pregnancy and tried to detect first degree atrioventricular block (AVB) using accurate echocardiographic tools, assuming they may characterize the initiation of the immune damage to the A-V conduction system, at which point the process might still be reversible. Some of the groups treated fetuses with first degree AVB with maternal oral fluorinated steroids. We summarized the results of all groups, including our group. We describe a case of a fetus that developed CAVB 6 days after normal sinus rhythm (NSR), who under aggressive dexamethasone therapy gradually reverted to NSR. This fetus had a previous sibling with CAVB. We assumed the immune damage to the conduction system in this small group of fetuses with a previous CAVB sibling may have occurred more quickly than usual. We therefore recommend a twice-weekly follow-up with these fetuses

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.

The output settings of echocardiographic systems should be set to the full (original) frame rate and lossless compression (e.g., run-length encoding) in order to transmit echocardiographic videos so that they retain their original quality. In addition, monitors and display cards of echocardiography systems and workstations should be able to support an adaptive refresh rate for displaying video at an arbitrary frame rate, including a high frame rate (90+ fps) without dropping frames and preserving the original frame duration. Currently, the only available option for echocardiography monitors is 144–165 Hz (or higher) based on adaptive frame rate G-Sync or FreeSync technology monitors. These monitors should be accompanied by compatible display cards. Echocardiography systems and workstation video playback software should support G-Sync or FreeSync adaptive frame rate technology to display echocardiography videos at their original frame rates without the effects of jitter and frame drops. Echocardiography systems should support an online display of the videos on the workstations during acquisition with the original quality. The requirements for web-based workstations are the same as for desktops workstations. Hospital digital networks should provide transmission and long-term archiving of the echocardiographic videos in their original acquisition quality.

Background: Rheumatic mitral stenosis (MS) is a relatively rare diagnosis in the developed countries and its treatment during pregnancy is challenging due to hemodynamic changes. With the demographic changes due to recent waves of immigration an increase in the prevalence of rheumatic heart disease is expected.

Objective: To evaluate maternal and neonatal complications in patients with mitral stenosis.

Methods: During the years 2006–2017, 22 women who underwent 31 pregnancies were followed at the Sheba Medical Center in Israel. We collected on regarding hemodynamic changes and their clinical course. MS was classified as mild, moderate, or severe according to mitral valve area by echocardiography. Maternal and fetal adverse events were evaluated according to severity of MS and compared by Poisson regression modeling.

Results: MS was severe in 7 pregnancies (22.6%), moderate in 9 (29%), and mild in 15 (48.4%). Twenty patients were managed conservatively and 2 underwent a successful percutaneous mitral balloon valvuloplasty (PBMVP) during pregnancy. All pregnancies ended with a liveborn neonate and with no maternal mortality. Peak and mean mitral pressure gradients increased during pregnancy from 13.3 ± 5.3 to 18.6 ± 5.1 mmHg and from 5.9 ± 2.3 to 9.6 ± 3.4 mmHg respectively (P < 0.05). Eight pregnancies (25.8%) were complicated by pulmonary congestion, 2/15 (13.3%) with mild MS, 2/9 (22.2%) with moderate, and 4/7 (57.1%) with severe MS. The adverse event rate was higher among patients with severe MS compared with moderate and mild MS [hazard ratio (HR) 3.15, 95% confidence interval (95%CA) 1.04–9.52 and HR 4.06, 95%CI 1.4–11.19 respectively, P < 0.05]. Nine of 31 deliveries were vaginal; 6 of 22 cesarean sections (27.3%) were performed for cardiac indications.

Conclusions: The number of total adverse events were higher among patients with severe MS. Patients with moderate and mild MS should be treated attentively, but good obstetric and maternal outcome can be expected.

Background: A cardiac restrictive filling patterns are associated with unfavorable prognoses. Cardiac interventions may change the natural history of patients.

Objectives: To investigate the prevalence of restrictive filling pattern in routine echocardiographic examinations and their association with morbidity and mortality.

Methods: The clinical and echocardiographic data of patients with newly diagnosed restrictive filling pattern were analyzed and summarized.

Results: Among 8000 patients who underwent an echocardiographic examination in our hospital in 2013, a restrictive filling pattern was identified in 256. Of these, 134 showed a restrictive filling pattern that was newly diagnosed. Mean age was 69 years. Hypertension, diabetes, and ischemic heart disease were found in 81%, 60%, and 53%, respectively. Left ventricular ejection fraction was 42% ± 16%. Severe valvular abnormalities were found in 18%. During follow-up (29 ± 15 months), 40% of patients died. The strongest predictor of mortality (73%) was moderate or more advanced aortic stenosis, P = 0.005. Renal failure was an important independent predictor of mortality (53%, P < 0.05). A very high E/E' ratio ≥ 20, was another independent mortality predictor (50%, P < 0.03). Patients who died were less likely to have undergone cardiac interventions than those who survived (26% vs. 45%, P < 0.03).

Conclusions: Prevalence of restrictive filling among echocardiographic studies is 3.2%. In a half of these, the restrictive filling pattern is a new diagnosis. Patients who are diagnosed with a new restrictive filling pattern have higher mortality rates. Patients with restrictive filling should be evaluated thoroughly for possible coronary artery or valvular heart disease.

Background: The search for the presence of vegetations in patients with suspected infective endocarditis is a major indication for trans-esophageal echocardiographic (TEE) examinations. Advances in harmonic imaging and ongoing improvement in modern echocardiographic systems allow adequate quality of diagnostic images in most patients.

Objectives: To investigate whether TEE examinations are always necessary for the assessment of patients with suspected infective endocarditis. 

Methods: During 2012–2014 230 trans-thoracic echo (TTE) exams in patients with suspected infective endocarditis were performed at our center. Demographic, epidemiological, clinical and echocardiographic data were collected and analyzed, and the final clinical diagnosis and outcome were determined. 

Results: Of 230 patients, 24 had definite infective endocarditis by clinical assessment. TEE examination was undertaken in 76 of the 230 patients based on the clinical decision of the attending physician. All TTE exams were classified as: (i) positive, i.e., vegetations present; (ii) clearly negative; or (iii) non-conclusive. Of the 92 with clearly negative TTE exams, 20 underwent TEE and all were negative. All clearly negative patients had native valves, adequate quality images, and in all 92 the final diagnosis was not infective endocarditis. Thus, the negative predictive value of a clearly negative TTE examination was 100%.

Conclusions: In patients with native cardiac valves referred for evaluation for infective endocarditis, an adequate quality TTE with clearly negative examination may be sufficient for the diagnosis.

 

Background: Contemporary therapies improve prognosis and may restore left ventricular (LV) size and function.

Objectives: To examine the prevalence, clinical features and therapies associated with reverse remodeling (RR) in dilated cardiomyopathy (DCM).

Methods: The study group comprised 188 DCM patients who had undergone two echo examinations at least 6 months apart. RR was defined as increased LV ejection fraction (LVEF) by ≥ 10% concomitant with ≥ 10% decreased LV end-diastolic dimension.

Results: RR occurred in 50 patients (26%) and was associated with significantly reduced end-systolic dimension, left atrial size, grade of mitral regurgitation, and pulmonary artery pressure. NYHA class improved in the RR group. RR was less common in familial DCM and a long-standing disease and was more prevalent in patients with prior exposure to chemotherapy. Recent-onset disease, lower initial LVEF and normal electrocardiogram were identified as independent predictors of RR. Beta-blocker dose was related to improved LVEF but not to RR. Over a mean follow-up of 23 months, 16 patients (12%) from the 'no-RR' group died or underwent heart transplantation compared to none from the RR group (P < 0.01).

Conclusions: Contemporary therapies led to an an improvement in the condition of a considerable number of DCM patients. A period of close observation while optimizing medical therapy should be considered before deciding on invasive procedures. 

Objectives: To assess the incidence and magnitude of the "athlete heart" phenomenon in an elite group of Israeli cyclists.

Methods: An echocardiography study was performed in 56 cyclists (49 males, mean age 38 ± 10 years, weekly average training 13.1 ± 5.9 hours); 96 sedentary subjects served as a control group.

Results: There were significant differences in left ventricular end-diastolic diameter (LVEDD) between cyclists and the control group (48 ± 4.7 mm vs. 45 ± 4.1 mm respectively, P < 0.001), as well as in inter-ventricular septum (IVS) thickness (9.9 ± 1.2 vs. 8.9 ± 1.2 mm, P < 0.001) and LV mass index (LVMI) (79 ± 16 vs. 68 ± 13 g/m², P < 0.001). In 5% of the cyclists LVEDD exceeded the upper normal limit of 56 mm. In 7% of the cyclists IVS thickness exceeded the upper normal limit of 11 mm. LV hypertrophy defined as LVMI ≥ 134 g/m² was absent in the entire cyclist group.

Conclusions: Endurance sport activity in well-trained Israeli sportsmen results in a modest increment in LV dimensions and LV mass. LV dilatation and wall thickness above values compatible with primary cardiac disease are rare. These results highlight that in Israeli athletes any abnormal echocardiographic value must be thoroughly investigated and not simply assumed to be a consequence of sport activities.

Background: Stentless aortic bioprostheses were designed to provide improved hemodynamic performance and potentially better survival.

Objectives: To report the outcomes of patients after aortic valve replacement with the Freestyle® stentless bioprosthesis in the Tel Aviv Medical Center followed for ≤ 15 years.

Methods and Results: Between 1997 and 2011, 268 patients underwent primary aortic valve replacement with a Freestyle bioprosthesis, 211 (79%) of them in the sub-coronary position. Mean age, Charlson comorbidity index and Euro-score were 71.0 ± 9.2 years, 4.2 ± 1.5 and 10.2 ± 11 respectively, and 156 (58%) were male. Peak and mean trans-aortic gradient decreased significantly (75.0 ± 29.1 vs. 22.8 ± 9.6 mmHg, P < 0.0001; and 43.4 ± 17.2 vs. 12.1 ± 5.4 mmHg, P < 0.0001 respectively) in 3 months of follow-up. Mean overall follow-up was 4.9 ± 3.1 years and was complete in all patients. In-hospital mortality was 4.1% (n=11) but differed significantly between the first 100 patients operated before 2006 and the last 168 patients operated after January 2006 (8 vs. 3 patients, 8.0% vs. 1.8%, P = 0.01). Overall, 5 and 10 year survival rates were 85 ± 2.5% and 57.2 ± 5.7%, respectively. Five year survival was markedly improved in patients operated after January 2006 compared to those operated in the early years of the experience (92.3 ± 2.3% vs. 76.0 ± 4.4%, P = 0.0009). All the 21 octogenarians operated after January 2006 survived surgery, with excellent 5 year survival (85.1 ± 7.9%). Six patients required reoperation during follow-up: structural valve deterioration in five and endocarditis in one.

Conclusions: Aortic valve replacement with the Freestyle bioprosthesis provides good long-term hemodynamic and clinical outcomes, even in octogenarians. Valve calcification is the major (and rare) mode of valve deterioration leading to reoperation in these patients.