Rare cause of dynamic aortic obstruction in pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries: a case report

A 2-month-old infant with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries developed an aneurysmatic elongation of the tricuspid valve tissue that partially closed and dynamically protruded through the ventricular septal defect, beneath the aortic valve. This rare finding caused dynamic left ventricle outflow tract obstruction and recurrent cardiac arrests and ultimately required surgical intervention.

Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children

Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes.

Experience of stent implantation for recurrent aortic arch obstruction following Norwood or Damus–Kaye–Stansel operation over the last decade

Background:Recurrent aortic arch obstruction following the Norwood procedure is recognised as an important complication. Balloon arch angioplasty is associated with a high recoarctation rate.Methods:We sought to evaluate the prevalence and outcome of stent implantation for recoarctation in children following Norwood or Damus–Kaye–Stansel procedure over the past decade at a single national cardiology centre.Results:Of 114 children who underwent Norwood procedure or Damus–Kaye–Stansel procedure between January 2003 and June 2013, 80 patients survived. Of these 15 children underwent stent implantation for recoarctation. Six of these patients had previous balloon angioplasty. The median age at stent implantation was 4.4 months (range 2–82 months). The median peak aortic arch gradient at catheterisation decreased from 26mmHg (range 10–70mmHg) to 2mmHg (range 0–20mmHg). The median luminal diameter increased from 4.7 mm (range 3.2–7.9 mm) to 8.6 mm (range 6.2–10.9 mm). The median coarctation index increased by 0.49 (range = 0.24–0.64). A Valeo stent was employed in 11 children, a Palmaz Genesis stent in 2 patients, a MultiLink stent in 1 child, and a Jomed covered stent in 1 child. Two factors were associated with the need for stent placement: previous arch angioplasty (p valve < 0.001, χ-square 11.5) and borderline left ventricle (p = 0.04, χ-square = 4.1). Stent migration occurred in one child. There were two deaths related to poor right ventricular systolic function and severe tricuspid regurgitation. Six patients underwent redilation of the stent with no complications.Conclusions:The prevalence of recurrent aortic arch obstruction following Norwood/Damus–Kaye–Stansel procedure was 18%. Stent implantation is safe and reliably eliminates the aortic obstruction. Redilation can be successfully achieved to accommodate somatic growth or development of stent recoarctation.

Technical features of Norwood procedure as the most important factor, affecting the outcome of treatment of single-ventricle patients with obstructed systemic output

Theobjectivewas to improveNorwood procedure outcomes by discovering optimal surgical technique and perfusion strategy.Material and methods.Results of treatment of single-ventricle patients with obstructed systemic output during 2005–2017 were analyzed (64 patients). 61 patients underwentNorwood procedure. Outcomes were compared between groups with different perfusion strategies and different methods of aortic arch reconstruction.Results.Mortality in two-regional perfusion group was 23 %, in group of circulatory arrest and selective cerebral perfusion – 53 % (p=0.020). Early aortic obstruction was a risk factor of mortality (p=0.024). Median time of freedom from obstruction of aorta was 3 days in xenopericardium group, 76 days in pulmonary allograft group, and 390 days in isthmus resection and pulmonary allograft group (p=0.002).Conclusion.Two-regional perfusion and aortic arch repair with pulmonary allograft patch after isthmus resection are our methods of choice as they allow to reduce the incidence of complications afterNorwood procedure.

Left aortic arch with right descending aorta and severe coarctation: an unusual “vascular clamp” with airway compression

Left aortic arch with right descending aorta is a rare congenital anomaly. We describe the clinical presentation of this unusual anomaly associated with cardiorespiratory compromise from severe aortic obstruction and left main bronchus compression. The anatomical peculiarities, embryological basis, and surgical solutions are presented.