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.

Right Superior Vena Cava Connected Left Atrium with Partial Anomalous Pulmonary Venous Return and Intact Atrial Septum: An Unusual Cause of Paradoxical Embolism.

A six-month-old girl presented with history of convulsion and cyanosis, was eventually found to have a right superior vena cava connected to left atrium with anomalous upper right pulmonary vein return and without atrial septal defect. Diagnosis was confirmed by cardiac magnetic resonance imaging. The patient underwent a successful cardiac surgery correction, routing SVC to right atrium and repair of right upper pulmonary vein to left atrium.

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 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.