Journey to the center of the heart: Bilateral absent superior vena cava with heart block.

Bilaterally absent superior vena cava (SVC) is extremely rare anomaly with a few case reports in the literature. Without associated congenital cardiac disease, these anomalies are asymptomatic. This report describes an adult patient with bilaterally absent SVC presenting with Mobitz type II heart block and a structurally normal heart.

Pericardial effusion in prosthetic and native valve infective endocarditis.

BACKGROUND: Pericardial effusion (PE) is widely believed to signify more advanced infective endocarditis (IE) and a generally worse outcome. PURPOSE: To determine the prevalence and clinical significance of PE in a series of patients with confirmed native and prosthetic valve infections. METHODS: Data were collected from 338 consecutive patients with definite or possible IE who visited a single referral center; these patients were examined for the presence of PE as detected by transthoracic echocardiography. Clinical characteristics, the incidence of complications, and outcomes were compared between patients with IE with and without PE. IE patients with PE were then divided into two subgroups: those with and those without cardiac prostheses. RESULTS: Eighty-eight patients out of the total 338 (26%) were found to have PE. Compared with patients who did not have PE, patients who did were significantly younger (32.9 ± 13.4 vs 29.0 ± 9.2, p = 0.003), had more left-sided vegetation (55.6% vs 77.3%, p < 0.001), more root abscesses (9.2% vs 25.0%, p < 0.001), needed surgery more frequently (68.0% vs 84.1%, p = 0.001), and had a higher mortality rate (22.0% vs 32.9%, p = 0.03). PE was not found to be a predictor of mortality. No significant difference was found between IE patients with PE with (n = 13) and without (n = 75) prostheses with regard to causative organisms, clinical characteristics, or clinical outcomes. CONCLUSION: Regardless of whether the IE was in native or prosthetic valves, compared with patients without PE, patients with PE had more severe infections and a worse prognosis, but PE was not an independent predictor of mortality.

Adult congenital heart disease registry at Cairo University: a report of the first 100 patients.

AIMS: To establish a clinical registry for adult patients with congenital heart disease (CHD) managed in Cairo University Hospitals, aiming at description of the pattern and clinical profile of such patients. METHODS: Patients were recruited from both Cardiovascular Medicine Department Outpatient Clinic and inpatient wards of Cairo University Hospitals. Clinical data were collected from hospital records and directly from patients by treating cardiologists. Collected data were then registered in a dedicated database system and subsequently analyzed. RESULTS: Patients (49% males) ranged in age from 16 to 63 years, with a median of 25 years. Fifty-one patients were in the age-group from 20 to 30 years, with only 9% aged 50 years or older. Seventy-eight patients had acyanotic lesions, with atrial septal defect being the most common primary diagnosis (20% of total lesions). The remaining 22 patients had cyanotic heart disease, with tetralogy of Fallot being the predominant diagnosis (45% of cyanotic lesions). Six patients presented with infective endocarditis in the setting of CHD. Four women (8% of females) presented during pregnancy. Forty-six patients were sent for surgical correction/repair, while percutaneous intervention was planned in 20 patients. CONCLUSIONS: A new registry of adult patients with CHD managed in Cairo University Hospitals provides useful information, including the extent to which congenital heart defects are underdiagnosed and undertreated during infancy and childhood. In addition, those who were previously treated early in life require long-term follow-up in specialized centers. Establishment of a multidisciplinary team with expert physicians (cardiologists, dentists, obstetricians, and psychiatrists), cardiac surgeons, and nurses may be facilitated by development of a dedicated database system. Continuous financial support is a major challenge.

Comparison of three-dimensional echocardiographic software packages for assessment of left ventricular mechanical dyssynchrony and prediction of response to cardiac resynchronization therapy.

AIMS: We directly compared TomTec and QLAB software packages for the three-dimensional echocardiographic (3DE) assessment of left ventricular (LV) dyssynchrony including their ability to predict response to cardiac resynchronization therapy (CRT) in patients with ischaemic and non-ischaemic cardiomyopathy. METHODS AND RESULTS: A total of 140 heart failure patients with the LVEF ≤35% and 60 healthy volunteers underwent 3DE. A subgroup of 60 patients underwent CRT and were evaluated before and 6-12 months after implantation. The systolic dyssynchrony index (SDI) was derived from the dispersion of time to minimum regional volume for all 16 LV segments and measured with both software packages and compared using Pearson's correlation and Bland-Altman analysis. Measurements of SDI were significantly higher using TomTec compared with QLAB in both patients (10.9 ± 3.8 vs. 9.7 ± 3.9, P < 0.001) and healthy volunteers (4.1 ± 0.8 vs. 2.4 ± 1, P < 0.001), with large biases and wide limits of agreement. A moderate correlation (r = 0.65, P < 0.001) was observed between both software packages in patients while their inter-observer and intra-observer reliability were good. Of the 60 patients undergoing CRT, reverse remodelling as a measure of response was observed in 41 patients (68%). The optimal SDI cut-off value to predict response to CRT was higher for TomTec than for QLAB (8.8 vs.7.3%, P < 0.001) and demonstrated better sensitivity and specificity (93 and 61%, respectively) compared with QLAB (88 and 33%, respectively). Response prediction in patients with non-ischaemic cardiomyopathy was excellent with a sensitivity and specificity of 95 and 100% for TomTec and 70 and 83% for QLAB using similar cut-off values of 9.1 and 9.2%, respectively. CONCLUSION: Different 3DE software packages for the assessment of mechanical dyssynchrony should not be used interchangeably until better software standardization is achieved. Dyssynchrony assessment with 3DE for the prediction of response to CRT seems particularly useful in patients with non-ischaemic cardiomyopathy.

A meta-analysis of left ventricular dyssynchrony assessment and prediction of response to cardiac resynchronization therapy by three-dimensional echocardiography.

AIMS: In a time of controversy regarding the use of echocardiography for assessment of left ventricular (LV) dyssynchrony and prediction of response to cardiac resynchronization therapy (CRT), this meta-analysis aimed to evaluate the feasibility and reliability of LV dyssynchrony assessment by three-dimensional echocardiography (3DE), determine clinically useful reference values in healthy subjects and heart failure patients, and examine the accuracy of 3DE to predict response to CRT. METHODS AND RESULTS: A total of 73 studies that evaluated the assessment of LV dyssynchrony by 3DE were eligible. The systolic dyssynchrony index (SDI) for 16 segments, being the predominant 3DE dyssynchrony parameter, was used for data pooling. Results demonstrated that LV dyssynchrony assessment by 3DE is feasible in 94% of studied subjects [95% confidence interval (CI): 92-95%)]. Pooled estimates of intraclass correlation coefficients (ICC) and limits of agreement (LoA) demonstrated that SDI has good interobserver (ICC: 0.92, LoA: 4.07%) and intraobserver reliability (ICC: 0.95, LoA: 2.10%). Reference values of SDI in healthy subjects, heart failure patients in general, and patients eligible for CRT were 2.7 ± 0.9%, 9.8 ± 3.9%, and 10.7 ± 3.6%, respectively. Meta-regression analysis demonstrated that reference values of SDI in healthy subjects significantly differed between different software [1.80% (95% CI: 0.55-3.05%), P< 0.001]. In patients eligible for CRT, SDI had good accuracy to predict treatment response with a weighted mean cut-off value of 9.8% and pooled estimates for sensitivity and specificity of 93% (95% CI: 89-97%) and 75% (95% CI: 58-93%), respectively. CONCLUSION: 3DE is a feasible and reliable tool for assessment of LV dyssynchrony and may have additional value to current selection criteria for accurate prediction of response to CRT.