Determination of Sensitivity and Specificity of High Resolution CT Scan in Evaluation of Cardiac and Extra-Cardiac Anomalies among Bangladeshi Children

Congenital heart disease (CHD) is one of the important causes of morbidity and mortality in the pediatric age group. Invasive angiography is considered as gold standard method for the diagnosis of pediatric cardiovascular diseases. Multi-detector CT cardiac angiogram by using 64 slice scanner is a useful modality and suitable alternative to previous invasive procedure in evaluation of congenital heart disease. The present study was conducted in the Department of Cardiology of NICVD during the period of September 2019 to August 2020 (One year). We selected 40 patients presenting in the outpatient department of National Institute of Cardiovascular Diseases and Hospital (NICVD) who were diagnosed to have congenital heart disease clinically and radiologically. CT angiogram revealed ASD in 10%, coarctation of aorta in 5%, Pentalogy of Fallot in 2.5%, Tetralogy of Fallot (TOF) in 32.5%, Ventricular septal defect (VSD) in 30.0%, PAH in 2.5%, PS in 5%, RVH with PS in 2.5%, PDA in 5% and Tricuspid atresia with ASD with VSD with PS in 2.5% patients. Conventional catheter angiogram revealed ASD in 5%, VSD in 32.5%, TOF in 30%, PS in 12.5%, Coarctation of aorta in 5% and Pentalogy of Fallots in 5%. The diagnostic performance of MDCT in the diagnosis and evaluation of congenital heart disease was high.

Right Heart Dysfunction in Adults With Coarctation of Aorta: Prevalence and Prognostic Implications

Background: Chronic elevation of left heart filling pressure causes pulmonary vascular remodeling, pulmonary hypertension, and right heart dysfunction. Although diastolic dysfunction is relatively common in patients with coarctation of aorta, there are limited data about the prevalence and prognostic implications of pulmonary hypertension and right heart dysfunction in this population. The purpose of the study was to assess right heart function and hemodynamics in patients with coarctation of aorta and to determine the relationship between right heart indices and cardiovascular events defined as heart failure hospitalization, heart transplant, or cardiovascular death. Methods: Right heart structure, function, and hemodynamics were assessed with these indices: right atrial volume, right atrial pressure, right atrial reservoir strain, right ventricular global longitudinal strain, right ventricular end-diastolic area, right ventricular systolic pressure, and tricuspid regurgitation severity. Right heart hemodynamic score, range 0 to 5, was generated based on the correlation between the right heart indices and cardiovascular events, using half of the cohort (derivation cohort, n=411), and then tested on the validation cohort (n=410). The goodness of fit and discrimination power was compared using C statistics and risk score. Results: The median follow-up in the derivation cohort was 8.2 (4.0–11.1) years, and 59 (14%) patients had cardiovascular events during this period. Right heart hemodynamic score was independently associated with cardiovascular events (hazard ratio, 1.64 [95% CI, 1.38–2.17]) for every unit increase in right heart hemodynamic score after adjustment for clinical and echocardiographic indices (C statistic, 0.718 [95% CI, 0.682–0.746]). The right heart hemodynamic score was also independently associated with cardiovascular events in the validation cohort (C statistic, 0.711 [95% CI, 0.679–0.741]). The C statistic difference (0.007 [95% CI, 0.014–0.022]) and risk score (0.86 [95% CI, 0.54–1.17]) suggest a good model fit. Conclusions: The current study underscores the prognostic importance of right heart dysfunction in patients with coarctation of aorta and suggests that right heart indices should be used for risks stratification in this population.

Cardiac Remodeling and Disease Progression in Patients With Repaired Coarctation of Aorta and Aortic Stenosis

Background: Valvulo-arterial impedance (Zva) is used for assessment of left ventricular (LV) global pressure load in patients with aortic stenosis (AS) and impaired arterial compliance. Because patients with repaired coarctation of aorta (COA) have impaired arterial compliance, we hypothesized that COA patients with greater than or equal to moderate AS (AS-COA group) will have higher Zva, symptomatic progression, and cardiovascular events, as compared to non-COA patients with similar AS severity (AS group). Methods: Propensity matching (1:1) of 71 AS-COA and 71 AS patients based on age, sex, body mass index, and aortic valve mean gradient (cohort 1). Of 172 patients, 117 patients (AS-COA [n=62]; AS [n=55]) underwent aortic valve replacement, cohort 2. Cohort 1 was used to assess the relationship between preoperative Zva, cardiac remodeling, and symptomatic progression, while cohort 2 was used to assess the relationship between postoperative Zva, LV mass index regression (reduction in LV mass index after aortic valve replacement), and cardiovascular events. Results: The AS-COA group had higher Zva (4.2±0.6 versus 3.5±0.4 mm Hg/mL·m 2 , P <0.001), more advanced cardiac remodeling, and higher 5-year incidence of symptomatic progression (85% versus 51%, P <0.001). Preoperative Zva was independently associated with cardiac remodeling (r=0.66, P <0.001) and symptomatic progression (hazard ratio, 1.06 [1.02–1.10], per mm Hg/mL·m 2 increase in Zva). The AS-COA group had higher postoperative Zva (3.3±0.5 versus 2.4±0.4 mm Hg/mL·m 2 , P <0.001), less robust LV mass index regression at 1-year post–aortic valve replacement, and higher 5-year incidence of cardiovascular events. Postoperative Zva was independently associated with LV mass index regression (r=−0.46, P <0.001) and cardiovascular events (hazard ratio, 1.06 [1.02–1.10], per mm Hg/mL·m 2 increase in Zva). Conclusions: Adults with AS-COA had higher LV global pressure load, cardiac remodeling, symptomatic progression, and cardiovascular events as compared to non-COA patients with similar severity of AS. Zva can identify patients at risk for adverse outcomes, and perhaps should be used for risk stratification with regards to timing of aortic valve replacement.

Effect of coarctation of aorta anatomy and balloon profile on the outcome of balloon angioplasty in infantile coarctation

Objective Coarctation of the Aorta (CoA) is a relatively common cardiovascular disorder. The present study aimed to evaluate the effect of COA anatomy and high versus low-pressure balloons on the outcome of balloon angioplasty among neonates and infants. Methods In this retrospective study, the neonates and infants undergoing balloon angioplasty at Namazi hospital were enrolled. After balloon angioplasty, immediate data results were promptly recorded.Moreover, midterm echocardiographic information was collected via electronic cardiac records of pediatric wards and clinical and echocardiographic data at least 12 months after balloon angioplasty. Finally, data were analyzed using SPSS-20. Results In this study, 42 infants were included. The median age at the time of balloon angioplasty was 1.55 (range 0.1–12) months and 66.7% of the patients were male. The mean pressure gradient of coarctation was 38.49 ± 24.97 mmHg, which decreased to 7.61 ± 8.00 mmHg (P < 0.001). A high-pressure balloon was used in 27, and a low-pressure balloon was used in 15 patients. COA's pressure gradient changed 30.89 ± 18.06 in the high-pressure group and 24.53 ± 20.79 in the low-pressure balloon group (P = 0.282). In the high-pressure balloon group, 14.81% and in the low-pressure group, 33.33% had recoarctation and need second balloon angioplasty (p < 0.021). The infant with discrete coarctation had a higher decrease in gradient and lower recoarctation. Conclusion Recoarctation rate was lower in the high-pressure balloon. The infant with discrete COA had a better response to the balloon with more decrease in gradient and lower recoarctation rate. Therefore, the stenotic segment anatomy needs to be considered in the selection of treatment methods.

Right Atrial Thrombus Associated with Central Venous Catheter after Surgical Repair of Co-arctation of Aorta

Central venous catheterization (CVC) is a routine and essential procedure performed in patients undergoing cardiac surgery. However, its use is not without complications ranging from mechanical to infectious to thrombotic in nature. Catheter-related right atrial thrombosis (CRAT) is usually underreported as it is commonly small in size and asymptomatic. Nevertheless, CRAT might lead to a potentially severe and life threatening complication in both adult and young patients. Up to 30% of patients with CVCs are estimated to be affected by CRAT. Anticoagulation and subsequently catheter removal were recommended as a first-line therapy and have suggested that no intervention may be associated with a high mortality rate. Here, we report a case of a 3-months-old male child who underwent coarctation of aorta repair and was diagnosed with right atrial thrombosis associated with CVC in postoperative period and anticoagulated with unfractionated heparin. The thrombus was followed up closely and when there was no further decrease in the size of the mass around 9 days after the diagnosis, the CVC was removed. The heparin was continued even after removal of CVC for further 7 days and patient was eventually discharged from the hospital.

Surgical outcome of Yasui procedure for preserving biventricular function: single centre experience

Background: Yasui procedure is surgical repair intended to preserve biventricular function for patients with left ventricle outflow tract obstruction associated with aortic arch lesions and ventricular septal defect. Methods: Retrospective chart review analysis of all patients who had Yasui procedure (2008–2020) comparing midterm outcome of one versus two stage repair. Results: Twenty patients (70% female) underwent Yasui procedure in our center. Eight patients (40%) had left ventricle outflow tract obstruction /interrupted aortic arch, 7 patients (35%) had left ventricle outflow tract obstruction /coarctation of aorta, 3 patients (15%) had double outlet ventricle and ventricular septal defect that were unattainable for tunneling to one of the semilunar valves without creating obstruction, and 2 patients (10%) had aortic atresia with hypoplastic aortic arch. All patients had associated ventricular septal defect. Fifteen patients (75%) had one-stage repair and 5 patients (25%) had two-stage repair. Means age and weight for one and two-stage repair were 1.3 ± 2 months, 13.4 ± 11.5 months and 3.3 ± 0.6 kg, 7.8 ± 3.4 kg, respectively. During follow up, 8 patients (40%) required re- intervention, mainly for right ventricle–pulmonary artery conduit either dilation or replacement. The average duration of follow up was 5 years with nil mortality. Conclusion: Yasui procedure is effective approach for children who have left ventricle outflow tract obstruction associated with aortic arch anomalies and ventricular septal defect. Survival rate with single or staged repair is comparably good. During the first 5 year of follow up, nearly 40% of operated patients required re-intervention.

Speckle-Tracking Global Longitudinal and Regional Strain Analysis in Neonates with Coarctation of Aorta: A Case-Control Study

Our objectives are to compare speckle-tracking peak global longitudinal (pGLS) and regional strain values in neonates with coarctation of aorta (CoA) and control groups. Echocardiographic parameters measured by speckle-tracking were studied in a retrospective single-center study. A comparison of pGLS and segmental deformation between neonates with CoA and control group was performed using a three-way mixed ANOVA model. There was a significant difference in the means of segmental strain values between CoA and control group at the apical (p = 0.018) and basal segments (p = 0.031) of the interventricular septum and at the apical segment (p = 0.026) of the left ventricle (LV). After correcting for multiple comparisons, the results had a tendency toward statistical significance (adjusted-p < 0.10). There was significant difference in the mean values of pGLS [F(1, 39) = 7.61, p = 0.009, adjusted p = 0.018] between the studied groups. The results of ROC analysis showed that a cut-off value of −16.60% for pGLS provided an estimated sensitivity of 92.31% (95% CI: [63.97, 99.81]) and 71.43% specificity (95% CI: [51.33, 86.78]) for the diagnosis of CoA in neonates (AUC = 0.794, 95% CI: [0.66, 0.93]). pGLS can be regarded as a feasible and reproducible parameter reflecting LV dysfunction in newborns with CoA when compared to newborns with a false-positive diagnosis.

Persistent hypertension and left ventricular hypertrophy after repair of native coarctation of aorta in adults

Background There are limited data about outcomes after repair of native coarctation of aorta (COA) in adulthood. The purpose of this study was to describe the procedural outcomes, hemodynamic improvement, regression of LV hypertrophy and cardiovascular events in adults undergoing repair of native COA. Methods The primary outcomes were procedural complications, re-interventions, and hemodynamic improvement (residual COA gradient &lt;20 mmHg) after COA repair. The secondary outcomes were improvement in the severity of hypertension, regression of left ventricular mass index (LVMI), and incidence of cardiovascular events (atrial fibrillation, heart failure hospitalization, and cardiovascular death) after COA repair. Clinical and imaging indices were assessed pre-intervention, and at 1-year (Y1) and 3-years (Y3) post-intervention. Results A total of 172 patients (age 38 [27–48]) underwent COA repair (surgical 161; transcatheter 11). There were no procedural deaths, and all patients had residual COA gradient &lt;20 mmHg. One patients that received transcatheter stent therapy required re-dilation of stent at 12 months post-intervention. There as a reduction in the prevalence of patients requiring anti-hypertensive therapy from 73% (pre-intervention) to 59% and 64% at Y1 and Y3 respectively. However, 72% and 69% of the patients still had systolic blood pressure &gt;130 mmHg (stage 1 and 2 hypertension) at Y1 and Y3 respectively. As compared to patients without hypertension at Y1, patients with stage 1 and stage 2 hypertension had less robust LVMI regression (% change in LVMI 12±5% vs 9±6% vs 5±3%, p&lt;0.001). Hypertension (HR 1.16, 1.05–1.27) and LVMI regression (HR 0.86, 0.81–0.90) were independently associated with cardiovascular events. Conclusions Persistent hypertension was common after repair of native COA in adults. Hypertension (including stage 1 hypertension) was associated with suboptimal regression of LVMI and cardiovascular events. These results are concerning, and highlight the importance of early COA diagnosis and repair, and optimal medical therapy for hypertension after COA repair. FUNDunding Acknowledgement Type of funding sources: None.