Progressive changes in residual gradient after aortic coarctation repair and its role in the prediction of reintervention: A longitudinal data analysis.

Background: Repair of aortic coarctation through left thoracotomy is the standard treatment when anatomically feasible. Long-term outcomes are well studied, including the need for reintervention. However, the timely variation in residual gradients across the repaired segment is ill-defined. The aim of this work was to study the progressive changes of estimated peak gradient (ePG) acquired by transthoracic continuous-wave Doppler echocardiography across the aortic arch after repair and to assess the role of timing of assessment and values of ePG in prediction of reintervention. Materials and Methods: All eligible patients for this study who underwent aortic coarctation repair through left thoracotomy from 2001 to 2017 were reviewed. Details of the aortic arch dimensions and associated lesions were obtained by transthoracic echocardiography (TTE). The primary outcome was the ePG across the aortic arch after repair. Longitudinal data analyses with mixed effect modeling were used to determine independent predictors for ePGs. Results: A total of 312 patients were included. Median age and weight were 30 days and 4 kg, respectively. Associated lesions included ventricular septal defect (VSD) (53%), bicuspid aortic valve (53%) and mitral stenosis (25%). Over 15-years follow-up the freedom from reintervention was 92.3%, while 24 out of the 312 patients underwent reintervention (7.7%). Longitudinal data analyses of serial 2566 TTE studies were done. The graphical display showed that the ePG across coarctation area in the first postoperative TTE was the most notable difference between those who underwent reintervention and those who did not. Further testing with proportional hazard and logistic regression modeling confirmed this finding. The area under receiver operating curve statistics showed that an ePG of 25 mmHg is an optimal cutoff value for the prediction of the reintervention. Conclusions: The ePG acquired in the first postoperative TTE is the most important predictor for reinterventions. The presence of VSD is associated with decreased ePGs. We propose that an ePG in the first postoperative TTE of 25 mmHg or more is a strong predictor for the need of reintervention.

Outcome of ventricular septal defect closure with the Nit-Occlud® Le VSD-Coil: single centre experience.

The objective of this study was to review the outcome of patients who underwent transcatheter closure of ventricular septal defect (VSD) using Nit-Occlud® Leˆ VSD-Coil.A retrospective study was carried out at King Abdulaziz Cardiac Center on all paediatric patients who underwent interventional VSD closure, during the period from 2011 and 2016. Data were collected, including demographic information, cardiac diagnosis, pre-operative diagnosis, intra-operative findings and postoperative events. Out of 46 patients, who were evaluated for VSD device closure, 17 of them were excluded; 24 patients out of 29 had successful closure of VSD using Nit-Occlud Leˆ VSD-Coil (success rate of 83%), and five cases were referred to surgery. The age group was from 2 years up to 18 years. Almost 90% of VSD was the perimembranous (PM) type (26 patients) and 3 patients (10%) with muscular VSD. Ten of the 26 PM VSDs had TV accessory tissue partially covering the VSD defects. The hemodynamic assessment showed pulmonary blood flow to systemic blood flow ratio (Qp:Qs) > 1.5 in almost all the patients who underwent defect closure. Immediate closure of the defect was achieved in 11/24 patients (45%). In five additional patients, there was a tiny residual and eight with a small residual. With 6 months of follow-up, only six patients remained with tiny residual VSD defects.The Nit-Occlud® Leˆ VSD-Coil device has minimal effects on the aortic and tricuspid valves, and there was no permanent atrioventricular block, especially in patients with PM VSDs. Patients with a residual shunt should be followed, especially for haemolysis.

Outcome of truncus arteriosus repair: 20 years of single-center experience comparing early versus late surgical repair.

BACKGROUND: Truncus arteriosus is a rare CHD. Neonatal and early infancy repair is recommended though some cases may present late. The aim of our study is to investigate the current results of truncus arteriosus repair and to analyse the differences in outcome and reintervention need between early versus late truncus arteriosus surgical repair. MATERIAL AND METHODS: In this cohort study, we reviewed all children who underwent truncus arteriosus repair from 2001 till 2021. We divided patients into two groups; early repair group including patients repaired at age less than 3 months and late repair group including patients who had repair at 3 months of age and later. We compared both groups for outcome variables. RESULTS: Sixty-four children had truncus arteriosus repair including 48(75%) patients in early repair and 16(25%) patients in late repair groups. Peri-operative course was comparable between both groups. Post-surgery, we observed pulmonary hypertension in 6(12%) patients in early repair group comparing with 11(69%) patients in late repair group (p = 0.0001). In the last follow-up visit, pulmonary hypertension resolved in all early repair group patients while 6(37.5%) patients in late repair group continued to have pulmonary hypertension (p = 0.0001). Twenty-three(36%) patients required reintervention including 22(48%) in early repair group versus 1(6%) in late repair group (p = 0.007). CONCLUSION: In general, the outcome of early truncus arteriosus repair is excellent with resolution of pulmonary hypertension following early repair. Late repair caries higher risk of persistent pulmonary hypertension (37.5%). About one-third of the patients who had truncus arteriosus repair will require re-intervention within 38±38.4 months after initial surgery.

Pulmonary Vascular Resistance Measurement Remains Keystone in Congenital Heart Disease Management.

Pulmonary vascular resistance (PVR) plays a major role in congenital heart management and critical decision. The impact of pulmonary vascular disease in the early and late morbidity and mortality after cardiac surgery and interventional catheterization in congenital heart defect (CHD) highlights the importance of critical evaluation for PVR. Currently, PVR is evaluated with invasive cardiac catheterization for hemodynamic data collection, processing, and analysis. Despite the limitation of hemodynamic evaluation in the setting of CHD, accurate data analysis, and interpretation have significant impact on clinical outcome and procedure success. This article reviews the basic calculation of PVR in the setting of congenital heart disease with diagrammatic illustration for easy understanding of the hemodynamic.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. METHODS: In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. RESULTS: Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. CONCLUSIONS: PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Pulmonary arteriovenous malformation in a neonate: a condition commonly misdiagnosed.

Pulmonary arteriovenous malformations (PAVMs) are congenital defects in the form of an anomalous bridging between a pulmonary arterial and venous system that sidesteps the normal pulmonary capillary. This anomaly is usually associated with hereditary hemorrhagic telangiectasia, leftover small group are sporadic cases but may occur as an isolated anomaly or as multiple lesions. Rarely, such abnormalities can be acquired. Few cases are diagnosed in the neonatal period, as it will pass silent without symptoms. Clinical manifestations occur soon after birth as cyanosis, tachypnea, and an abnormal sound as bruit over the AVM; and the most important point is the clinical suspicion of diagnosis in absence of pulmonary parenchymal and cardiac disease that can explain the persistence of cyanosis. We report a successful Amplatzer vascular plug occlusion of isolated multiple left side PAVM in a neonate, which was done on two stages with a 6 months interval, with no complications. Over 2-year follow-up, the patient remained well with no symptoms.