PDA Closure in ELBW Infants: If, When, and How to Do It

Patent ductus arteriosus (PDA) is the most common cardiovascular condition afflicting premature neonates especially those born extremely low birth weight (ELBW). Despite five decades of scientific inquiry which has produced thousands of publications including over 65 randomized controlled trials, cardiologists, neonatologists, and surgeons still cannot answer simple questions such as if, when and how to close to the PDA in ELBW infants. This chapter will examine current evidence in order to answer these fundamental questions. The chapter will specifically focus on transcatheter PDA closure (TCPC), which albeit a new therapy, has displayed great potential to be the best therapeutic option in the future. It is about time that physicians from all sub-specialties come together and integrate the evidence to develop a management algorithm for ELBW infants with hemodynamically significant PDA

Cardiac Catheterization in Congenital Heart Disease

Interventional pediatric cardiology is a specialty of pediatric cardiology that deals specifically with the catheter-based treatment of congenital heart diseases. Cardiac catheterization involves the evaluation and manipulation of the heart and surrounding vessels through catheters place in peripheral vessels. In this chapter we begin by discussing the significant difference between adult and pediatric interventional cardiology. We will discuss basic hemodynamic measurements performed in cardiac catheterization and its application to congenital heart disease. Stent and balloon catheters are briefly discussed. Finally, specific catheter based interventional techniques, indications, and complications for various pediatric congenital heart disease is described

Aortic cusp extension valvuloplasty with or without tricuspidization in children and adolescents: Long-term results and freedom from aortic valve replacement

ObjectiveAortic cusp extension valvuloplasty is increasingly used in the management of children and adolescents with aortic stenosis or regurgitation. The durability of this approach and the freedom from valve replacement are not well defined. A study was undertaken to investigate outcomes.MethodsFrom July 1987 to November 2008, 142 patients aged less than 19 years underwent aortic cusp extension valvuloplasty in the form of pericardial cusp extension and tricuspidization (when needed). Three patients with truncus arteriosus and severe truncal valve insufficiency were excluded. From the available follow-up data of 139 patients, 50 had bicuspid aortic valves, 40 had congenital aortic valve stenosis, 41 had combined congenital aortic valve stenosis/insufficiency, and 8 had other diagnoses. Median follow-up was 14.4 years (0.1–21.4). Long-term mortality and freedom from aortic valve replacement were studied.ResultsThere were no early, intermediate, or late deaths. Z-values of left ventricular end-diastolic dimension, aortic annulus, aortic sinus diameter, and sinotubular junction diameter before aortic valve replacement were 4.2 ± 3.11, 2.3 ± 1.25, 4.4 ± 1.23, and 1.84 ± 1.28, respectively. During the follow-up period, 64 patients underwent aortic valve reinterventions. The Ross procedure was performed in 32 of 139 patients (23%) undergoing aortic cusp extension valvuloplasty. Other aortic valve replacements were undertaken after 16 aortic cusp extension valvuloplasties (11.5%). Freedom from a second aortic cusp extension valvuloplasty or aortic valve replacement at 18 years was 82.1% ± 4.2% and 60.0% ± 7.2%, respectively.ConclusionAortic cusp extension valvuloplasty is a safe and effective surgical option with excellent survival and good long-term outcomes in children and adolescents. The procedure provides acceptable durability and satisfactory freedom from aortic valve replacement

Acute and mid‐term outcomes of stent implantation for recurrent coarctation of the aorta between the Norwood operation and fontan completion: A multi‐center Pediatric Interventional Cardiology Early Career Society Investigation

ObjectivesWe sought to evaluate outcomes of stent implantation (SI) for recurrent coarctation of the aorta (RC) following the Norwood operation.BackgroundRC is common following the Norwood operation. Balloon angioplasty (BA) is standard treatment but may result in unsatisfactory relief of RC. SI may improve RC, but outcome data are limited.MethodsWe performed a multi‐center retrospective study of patients who underwent SI for RC between the Norwood operation and Fontan completion. Outcomes were examined, including procedural success, serious adverse events (SAE), and freedom from re‐intervention. A core laboratory was utilized to review angiograms. Coarctation Index (CI) was calculated before and after SI. Paired t‐test and Wilcoxon signed‐rank test were used to compare pre‐ and post‐SI variables.ResultsThirty‐three patients at 8 centers underwent SI for RC at a median age of 5 months (IQR 4.1, 13.3) and weight of 5.9 kg (5.2, 8.6). Aortic arch gradient improved from 20 (15, 24) to 0 (0, 2) mmHg following SI (P < 0.0001). The median CI improved from 0.54 (0.43, 0.62) to 0.97 (0.89, 1.06) following SI (P < 0.0001). There were no procedural deaths but SAEs occurred in 12 (36%) patients. During a median follow‐up duration of 29.7 months (6.8, 48.0), freedom from death or heart transplant was 82%, and from re‐intervention was 45%, with median time to re‐intervention of 20.1 months (11.4, 40.3).ConclusionsSI for treatment of RC in patients after the Norwood operation provides excellent acute relief of obstruction. Intraprocedural hemodynamic instability is common and re‐intervention is frequent at mid‐term follow‐up.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/140022/1/ccd27231_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/140022/2/ccd27231.pd

Systemic and pulmonary artery aneurysms in incomplete Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis that can cause coronary artery aneurysms (CAA) in up to 25% if left untreated. Rarely, aneurysms of systemic arteries of all sizes have been reported to occur. The incidence of systemic artery aneurysms (SAA) with typical KD can be as high as 2.2%. Incomplete KD with SAA is not well described. We report a case of a 12-year-old boy with incomplete KD, giant CAA, diffuse SAA, and pulmonary artery aneurysms (PAA). The patient presented with fever, malaise, abdominal pain, maculopapular, rash and cervical lymphadenopathy. Echocardiogram showed multiple giant CAA. By angiography, diffuse ectasia and aneurysms of most medium-sized arteries throughout the body were noted including the lobar pulmonary artery branches. Incomplete KD was suspected. The patient was treated with intravenous gammaglobulin, methylprednisolone, and high-dose aspirin. Due to the systemic vasculitis, he also received cyclophosphamide. The patient\u27s clinical symptoms improved. Anticoagulation with warfarin was maintained. The patient remains asymptomatic three years later but with progressive CAA and stable SAA. The PAA caused no symptoms and resolved after the acute phase. Incomplete KD can manifest with CAA and SAA. This is the first report of PAA associated with KD. Surveillance for PAA in KD must be considered.\u3c. Learning objective: A high level of clinical suspicion is required to diagnose KD in adolescent patients as it can manifest in an atypical fashion. Incomplete KD can manifest with giant CAA and SAA. Pulmonary artery aneurysms (PAA) have not been previously described in cases of KD. This is the first case report of atypical KD with PAA. Imaging of pulmonary arteries and its branches should be considered in patients with atypical KD with CAA and SAA.\u3e

Mycotic Pseudoaneurysm Complicating Stent Placement for Native Aortic Coarctation in a Child

Mycotic aneurysms of the aorta are uncommon life-threatening lesions. Sporadic cases have been reported in adults after surgical intervention for native and recurrent coarctation of the aorta. We report a case of a rapidly progressive, large mycotic pseudoaneurysm in a 13-year-old boy 3 months after a bare-metal stent was used to treat native coarctation. The boy presented with slurred speech, bacteremia, and systemic septic embolization. During an emergency operation, the pseudoaneurysm was resected, and the infected aorta was replaced with an interposition graft. This rare complication may occur early or late after stent placement for coarctation of the aorta

Acute and long-term effects of endovascular debanding of pulmonary arteries in a swine model

Objectives: The primary objective of this study was to demonstrate that pulmonary artery (PA) debanding via cardiac catheterization using balloon angioplasty is feasible and safe in swine. The secondary objectives were to determine the acute and long-term effects of this therapy. Design: This is a chronic survival experimental study in newborn swine. Background: PA bands are used in infants for transient palliation of congenital heart defects with excessive pulmonary blood flow. Although rare, if these defects should close spontaneously or become hemodynamically insignificant, a sternotomy and occasionally cardiopulmonary bypass may still be required for band removal. Alternatively, debanding could be accomplished through less invasive methods. Interventions: The main pulmonary artery was banded in three piglets, and the left pulmonary artery in five piglets via mini-thoracotomy at a mean weight of 2.5 kg. Following a threefold increase in weight, the piglets underwent PA debanding via balloon angioplasty. Four piglets were sacrificed to evaluate the acute effects. The remainder were followed to evaluate long-term effects. Histopathology was performed on all piglets. Outcome measures: Reintervention rates. Histopathologic consequences of high pressure balloon angioplasty used for PA debanding acutely and after reinterventions. Results: Debanding was performed at a mean weight of 8.1 ± 2.23 kg. The median preintervention gradient across the band was 18 mm Hg. Debanding was successful in all piglets. The median postintervention gradient was 3.5 mm Hg. All piglets in the long-term model required re-interventions for recurrent stenosis at mean weights of 26 ± 1.6 and 61 ± 3.2 kg. Histopathology demonstrated vessel wall injury in only one piglet. Conclusions: Endovascular PA debanding can be safely achieved in a swine model. Angioplasty following debanding may be necessary for recurrent stenosis. This catheter-based therapy may provide a less-invasive alternative to surgery

Femoral vein homograft as Sano shunt results in improved pulmonary artery growth after Norwood operation

Objective To evaluate differences in interstage growth of pulmonary arteries between use of polytetrafluoroethylene and femoral vein homograft as Sano shunt during stage-I Norwood palliation. Methods A retrospective review of all patients who survived to the second stage following Norwood-Sano operation at two institutions was performed. Either polytetrafluoroethylene or the valved segment of femoral vein homograft was used for construction of the Sano shunt. The size of pulmonary arteries was compared at pre-Glenn catheterisation. Results A total of 48 neonates with the diagnosis of hypoplastic left heart syndrome or its variants comprised the study population. Femoral vein homograft of 5-6 mm diameter was used in 14 and polytetrafluoroethylene graft of 5 mm was used in 34 patients. The two groups were comparable in terms of preoperative demographics and age at time of pre-Glenn catheterisation (3.9±0.7 versus 3.4±0.8 months, p=0.06). Patients who received femoral vein homograft demonstrated a significantly higher pre-Glenn Nakata index [264 (130-460) versus 165 (108-234) mm2/m2, p=0.004]. The individual branch pulmonary arteries were significantly larger in the femoral vein group (right, 7.8±3.6 versus 5.0±1.2, p=0.014; left, 7.2±2.1 versus 5.6±1.9, p=0.02). There were no differences in cardiac index, Qp:Qs, ventricular end-diastolic pressure or systemic oxygen saturations. Conclusions Utilisation of a valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit during Norwood-Sano operation confers better interstage growth of the pulmonary arteries. Further studies are needed to evaluate the impact of femoral vein homograft on single ventricle function