Transcatheter recanalisation for absent intra-pericardial pulmonary arteries.

Unilateral absence of intra-pericardial pulmonary artery is a rare congenital malformation. If untreated, it can lead to morbidity and mortality in adulthood. Early intervention and restoration of physiologic pulmonary blood flow is necessary. Transcatheter stenting as initial intervention has been rarely reported. We present transcatheter recanalisation and stenting of the obliterated ductus in two newborns with unilateral absence of intra-pericardial pulmonary artery with cross-sectional imaging, procedural details, angiography, and follow up to surgical repair. We believe that such procedure promotes ipsilateral pulmonary vasculature growth to facilitate unifocalization surgery at a later age.

Prominent Eustachian Valve and Atrial Septal Defect Presenting With Chronic Hypoxemia in a Teenager.

Prominent Eustachian valves, with obligate right-to-left shunts, have been reported as a cause of neonatal hypoxemia. This anomaly can present as an obstructive structure that inhibits antegrade flow through the tricuspid valve and furthermore contributes to right-to-left atrial shunting in the presence of a patent foramen ovale or atrial septal defect. This case highlights the evaluation and diagnostic workup for chronic hypoxemia in an adolescent female patient and considerations for percutaneous atrial septal defect closure.

Balloon angioplasty of bidirectional Glenn anastomosis.

OBJECTIVE: We aim to assess the safety and efficacy of the transcatheter balloon dilation of superior cavopulmonary anastomosis (SCPA). BACKGROUND: SCPA stenosis can lead to impaired pulmonary blood flow, hypoxemia and development of veno-venous collaterals with right-to-left shunt. Balloon dilation of SCPA has been rarely reported and follow-up information is lacking. METHODS: We performed a retrospective review of patients who underwent cardiac catheterisation and angioplasty of SCPA and reviewed patient's demographics, diagnosis, SCPA surgery and post-operative course, catheterisation haemodynamics, procedural technique, angiography, and the findings of follow-up catheterisation. RESULTS: Between 2008 and 2017, seven patients showed significant narrowing of SCPA and underwent balloon angioplasty, all of whom had undergone bidirectional Glenn (BDG). Indications for cardiac catheterisation included persistent pleural effusion, hypoxemia, and echocardiographic evidence of BDG stenosis or routine pre-Fontan assessment. Five patients had bilateral SCPA. The procedure was successful in all cases with increase in the stenosis diameter from a median of 3.3 mm (range 1.2-4.7 mm) to a median of 4.7 mm (range 2.6-7.8 mm). All patients had at least one follow-up cardiac catheterisation. Only one patient required repeat angioplasty at the 2.3-month follow-up with no further recurrence. Sustained results and interval growth were noted in all other cases during up to 29 months of follow-up. No adverse events were encountered. CONCLUSION: Based on our small series, balloon angioplasty of BDG stenosis is feasible and safe and appears to provide sustained improvement with interval growth and only the rare recurrence of stenosis.

Early Surgical Closure of Atrial Septal Defect Improves Clinical Status of Symptomatic Young Children with Underlying Pulmonary Abnormalities.

Elective closure of atrial septal defect (ASD) is usually recommended during preschool ages. However, ASD may contribute to deteriorating health in the presence of significant comorbidity and, thus, may need earlier closure. There is a lack of clarity regarding the indications for and outcomes after ASD closure in infancy and early childhood. We investigated the benefits and safety of surgical ASD closure in symptomatic patients under 2 years of age. Retrospective chart review was conducted in patients who underwent surgical ASD closure within the first 2 years of life. Of 31 symptomatic ASD patients, 22 had persistent respiratory symptoms, 24 failure to thrive, and 9 pulmonary hypertension. Overall, 26 patients (84.0%) showed clinical improvement after ASD closure, including improved respiratory status (17/22; 77.3%), resumption of normal growth (15/24; 62.5%), and resolution of pulmonary hypertension (7/7; 100%, 2 patients unable to assess postoperatively). Two medically complicated patients died a few months after surgery unrelated to surgical complications. Four out of 8 ventilator-dependent patients were weaned from mechanical ventilation within 1 month after ASD closure. Closure of ASD did not improve those patients with highly advanced lung disease and/or medically complex conditions including underlying genetic abnormalities. Surgical complications were uncommon. Postoperative hospital stay was 4 to 298 days (median 8 days). The majority of our patients demonstrated significant clinical improvement after ASD closure. Early ASD closure is safe and beneficial for symptomatic infants and young children with associated underlying pulmonary abnormalities, especially bronchopulmonary dysplasia.

Lymphatic Management in Single-Ventricle Patients.

Lymphatic complications in patients with single ventricle include plastic bronchitis, protein-losing enteropathy, and chylous pleural effusion are a source of significant morbidity and mortality with historically limited therapeutic options. Novel lymphatic imaging techniques such as intranodal lymphangiography, dynamic contrast enhanced magnetic resonance lymphangiography and liver lymphangiography have allowed visualization of the lymphatic system and discovery of the pathophysiological mechanism of these conditions. This mechanism includes the combination of 2 factors: increased lymphatic flow in patients with elevated central venous pressure and presence of the lymphatic anatomical variant that allows the lymph to flow in close proximity to the serous (pleural space in chylothorax) or mucosal (plastic bronchitis and protein losing enteropathy) surfaces. Novel minimally invasive lymphatic interventional techniques, such as thoracic duct embolization, interstitial embolization and liver lymphatic embolization have allowed the obliteration of these abnormal lymphatic networks, resulting in resolution of the symptoms. Further refinement of the imaging techniques and interventional methods have subsequently allowed better patient selection and improved long term outcome of these procedures.

Case report: hepatobronchial lymphatic communications in single ventricle patients as a pathophysiological mechanism of plastic bronchitis: diagnosis and treatment.

BACKGROUND: Plastic bronchitis is a rare but devastating complication in single ventricle patients after Fontan completion. Recent advances in dynamic contrast-enhanced magnetic resonance lymphangiogram demonstrate the typical pathophysiological mechanism of the thoracic duct leaking lymphatic fluid towards the bronchi resulting in intraluminal casts. This has been termed abnormal pulmonary lymphatic perfusion and has been successfully treated in 94% of patients with thoracic duct occlusion. However, in some cases, this aberrant flow is not identified and therefore no intervention is available. This case report identifies a newly discovered origin of abnormal lymphatic flow from the liver to the bronchi and the treatment of these patients. CASE SUMMARY: We report two cases of plastic bronchitis in single ventricle patients with no identified abnormal lymphatic pulmonary perfusion from the thoracic duct. Both patients underwent liver lymphangiogram and demonstrated aberrant flow from the hepatic lymphatic ducts to the bronchi. These were successfully occluded, and plastic bronchitis symptoms resolved in both cases. DISCUSSION: The recent discovery of the abnormal pulmonary lymphatic perfusion from the thoracic duct to the bronchi has allowed successful treatment of 94% of single ventricle patients with plastic bronchitis. The discovery of hepatobronchial lymphatic perfusion reveals an occult aetiology of plastic bronchitis and a second target for embolization and successful treatment.

Identifying Subclinical Coronary Abnormalities and Silent Myocardial Ischemia After Arterial Switch Operation.

The incidence of late coronary complications is reported around 8% after arterial switch operation (ASO) for d-transposition of the great arteries, but the affected patients are usually asymptomatic. Exercise stress test (EST) and myocardial perfusion imaging (MPI) are common non-invasive modalities to screen for silent myocardial ischemia, but their diagnostic reliability in children after ASO is unclear. We retrospectively reviewed asymptomatic patients following ASO with EST, MPI, and coronary imaging studies (CIS) and examined the reliability of each test in identifying abnormal coronary lesions responsible for myocardial ischemia. Thirty-seven asymptomatic patients (24 males; ages 12.7 ± 3.7 years) had EST, in which 27 and 33 patients also underwent MPI and CIS, respectively. Exercise capacity was comparable to the age- and sex-matched 37 controls. In seven patients with angiographically proven moderate to severe coronary abnormalities, only two patients had positive EST and/or MPI, and five patients were negative including one patient who later developed exercise-induced cardiac arrest due to severe proximal left coronary artery stenosis. Two patients with positive EST or MPI showed no corresponding coronary abnormalities by CIS. Occurrence of acquired late coronary abnormalities did not correlate with the original coronary anatomy or initial surgical procedures. There is no single reliable method to identify the risk of myocardial ischemia after ASO. Although CIS are regarded as a gold standard, multidisciplinary studies are essential to risk-stratify the potential life-threatening coronary lesions after ASO in children.

Transcatheter closure of patent ductus arteriosus using the AMPLATZER™ duct occluder II (ADO II).

OBJECTIVES: The study purpose is to evaluate the safety and efficacy of the ADO II device for closure of patent ductus arteriosus (PDA) in children. BACKGROUND: Transcatheter treatment of PDA has been evolving for 40+ years and is the treatment of choice. The AMPLATZER™ Duct Occluder (ADO) device was developed for larger diameter ducts and is not ideal in all PDAs. ADO II was developed for small to moderate-sized ducts. METHODS: This is a single-arm, multicenter study evaluating safety and efficacy of the ADO II device. Patients <18 years were screened for a PDA ≤5.5 mm in diameter and 3-12 mm in length. Right and left heart catheterization was performed, and hemodynamic data were obtained at the time of implant. The diameter of the left pulmonary artery (LPA) and descending aorta, and the presence of any pre-existing pressure gradients across the LPA or aortic arch were assessed at baseline and 6 months post-implant. RESULTS: A total of 192 patients were enrolled. The median implant time was 74 min. Median fluoroscopy time was 12 min. A retrograde (aortic) approach was used in 33% of procedures and demonstrated a statistically significant reduction in fluoroscopy time (P value = 0.0018) compared to an antegrade approach. The device was successfully implanted in 93% of patients, with complete closure in 98% of successful implantations. CONCLUSIONS: In this prospective study, the ADO II was safe and effective for closure of small to moderate PDAs. Implantation is simple and the ability for retrograde aortic delivery reduces procedure-related radiation exposure. © 2017 Wiley Periodicals, Inc.

The Medtronic Micro Vascular Plug™ for Vascular Embolization in Children With Congenital Heart Diseases.

OBJECTIVES: To describe the early multi-center, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) in children with congenital heart disease (CHD) undergoing vascular embolization. BACKGROUND: The MVP is a large diameter vascular occlusion device that can be delivered through a microcatheter for embolization of abnormal blood vessels. METHODS: A retrospective review of embolization procedures using the MVP in children with CHD was performed in 3-centers. Occlusion of patent ductus arteriosus using the MVP was not included. RESULTS: Ten children underwent attempted occlusion using the MVP. The most common indication to use the MVP was failed attempted occlusion using other embolic devices. Five, single ventricle patients (median age 3-years, median weight 14.9 kg) underwent occlusion of veno-venous collaterals following bidirectional Glenn operation. Three patients (Median age 8 years) underwent occlusion of coronary artery fistulae (CAF). Two patients (age 7 months and 1 year) underwent occlusion of large aorto-pulmonary collaterals. A 7-day-old child with a large CAF required 2 MVPs and an Amplatzer Vascular Plug (AVP-II) for complete occlusion. Occlusion of all other blood vessels was achieved using a single MVP. One MVP embolized distally in an 8-years-old child with a large CAF. There were no other procedural complications or during follow-up (median 9 months). CONCLUSIONS: The MVP is a new, large-diameter vascular embolization device that can be delivered through a microcatheter. It may play an important role in providing highly effective occlusion of abnormal vessels in children.

Initial clinical experience with the Medtronic Micro Vascular Plug™ in transcatheter occlusion of PDAs in extremely premature infants.

OBJECTIVES: To describe the early multicenter, clinical experience with the Medtronic Micro Vascular Plug™ (MVP) for the occlusion of patent ductus arteriosus (PDA) in premature infants. BACKGROUND: The MVP is a large diameter plug that can be delivered through a microcatheter for occlusion of abnormal blood vessels. METHODS: A Retrospective review of PDA embolization procedures performed in two centers using the MVP was performed. RESULTS: Fifteen premature infants underwent attempted PDA occlusion using the MVP. The gestational age and birth weight were 25.6 ± 2.5 weeks and 735 ± 251 g, respectively. The median weight and age at the time of the procedure were 1,210 g (700-3,500 g) and 4.5 weeks (2-12 weeks), respectively. Median procedure and fluoroscopy times were 45 and 6.5 min, respectively. The median radiation and contrast doses were 19.7 mGy and 2.4 mL/kg, respectively. Antegrade occlusion was successfully achieved in 13 patients <2 kg with only femoral venous access aided by echo guidance. The two patients >2 kg had arterial access and attempted retrograde occlusion; one of which was unsuccessful due to the PDA being short and wide. Complete closure was observed in 13 of 14 successful procedures (93%), with one patient having a small residual shunt that was not seen on follow-up. There were no complications related to the procedure or noted during follow-up (Median 11 months). CONCLUSIONS: The MVP is a new, large-diameter vascular embolization device that may be useful for the occlusion of PDA in extremely small, premature infants. © 2016 Wiley Periodicals, Inc.

Maladaptive aortic properties after the Norwood procedure: An angiographic analysis of the Pediatric Heart Network Single Ventricle Reconstruction Trial.

OBJECTIVES: Aortic arch reconstruction in children with single ventricle lesions may predispose to circulatory inefficiency and maladaptive physiology leading to increased myocardial workload. We sought to describe neoaortic anatomy and physiology, risk factors for abnormalities, and impact on right ventricular function in patients with single right ventricle lesions after arch reconstruction. METHODS: Prestage II aortic angiograms from the Pediatric Heart Network Single Ventricle Reconstruction trial were analyzed to define arch geometry (Romanesque [normal], crenel [elongated], or gothic [angular]), indexed neoaortic dimensions, and distensibility. Comparisons were made with 50 single-ventricle controls without prior arch reconstruction. Factors associated with ascending neoaortic dilation, reduced distensibility, and decreased ventricular function on the 14-month echocardiogram were evaluated using univariate and multivariable logistic regression. RESULTS: Interpretable angiograms were available for 326 of 389 subjects (84%). Compared with controls, study subjects more often demonstrated abnormal arch geometry (67% vs 22%, P < .01) and had increased ascending neoaortic dilation (Z score 3.8 ± 2.2 vs 2.6 ± 2.0, P < .01) and reduced distensibility index (2.2 ± 1.9 vs 8.0 ± 3.8, P < .01). Adjusted odds of neoaortic dilation were increased in subjects with gothic arch geometry (odds ratio [OR], 3.2 vs crenel geometry, P < .01) and a right ventricle-pulmonary artery shunt (OR, 3.4 vs Blalock-Taussig shunt, P < .01) but were decreased in subjects with aortic atresia (OR, 0.7 vs stenosis, P < .01) and those with recoarctation (OR, 0.3 vs no recoarctation, P = .04). No demographic, anatomic, or surgical factors predicted reduced distensibility. Neither dilation nor distensibility predicted reduced right ventricular function. CONCLUSIONS: After Norwood surgery, the reconstructed neoaorta demonstrates abnormal anatomy and physiology. Further study is needed to evaluate the longer-term impact of these features.

Coronary artery problems late after arterial switch operation for transposition of the great arteries.

BACKGROUND: The incidence of late coronary artery abnormalities after arterial switch operation (ASO) for d-loop transposition of the great arteries may be underestimated. METHODS AND RESULTS: We retrospectively reviewed coronary artery morphology in 40 of 97 patients who survived the first year after ASO. Seven asymptomatic patients developed significant late coronary artery abnormalities. One patient died suddenly at home with severe left coronary artery (LCA) ostial stenosis at age 3.8 years. The second patient collapsed during exercise at age 9.6 years due to ventricular fibrillation and severe LCA ostial stenosis despite prior negative exercise stress test (EST) and myocardial perfusion imaging (MPI). The third patient was found to have moderate ostial stenosis of the LCA with negative EST and MPI. The fourth patient with exercise-induced ST-T depression and myocardial perfusion defect was shown to have complete LCA occlusion with collateral vessel formation. Three other patients had complete proximal obliteration of either of the coronary arteries with collateral supply. An additional 4 asymptomatic patients had trivial-mild narrowing of the LCA on routine selective coronary angiogram. CONCLUSIONS: Incidence of late coronary stenosis or occlusion was not infrequent after ASO (11.3%) and presented usually without preceding symptoms and often after negative non-invasive screening. We advocate routine coronary imaging in all patients after ASO before they participate in competitive sports.

Improving early outcomes following hybrid procedure for patients with single ventricle and systemic outflow obstruction: defining risk factors†.

OBJECTIVES: The hybrid procedure has become an accepted management strategy for patients with single ventricle and systemic outflow obstruction (SVSOO), particularly those considered at high risk for mortality following Stage I Norwood. We sought to refine patient selection by identifying clinical variables associated with early mortality. METHODS: Single-centre review of all patients with SVSOO undergoing a hybrid procedure between January 2003 and December 2012. Demographic, clinical and outcome data were collected from the electronic medical record. The primary outcome was operative mortality (mortality within 30 days of the operation or prior to hospital discharge). RESULTS: Thirty-four patients underwent hybrid palliation at a median age of 5 days (IQR 3-8.5) and a median weight of 2.5 kg (IQR 2.2-2.9). Aortic atresia was present in 13 of 34 patients. Emergency banding for resuscitation was performed in 8 cases. Subsequent procedures included Stage I Norwood in 14, combined Stage I and II Norwood in 8, biventricular repair in 3 and cardiac transplantation in 2. Median cardiac intensive care unit length of stay was 54 days (IQR 27-92). Seven patients had no subsequent procedure. There were 11 operative deaths. Non-modifiable patient-related factors had a significant association with operative mortality, including weight under 2.0 kg (OR 18; 95% CI 1.8-188.3, P = 0.0008) and aortic atresia (OR 6.1; 95% CI 1.2-30.1, P = 0.026). Most significant was the interaction between aortic atresia and weight under 2.0 kg on early mortality (OR 13.1; 95% CI 1.9-92 P = 0.0039). The multivariate analysis demonstrated that the presence of aortic atresia (OR 13.4; 95% CI 1.2-151) and a birthweight of 2.0 kg or less (OR 51; 95% CI 2.4-999) were also significant predictors of operative mortality. CONCLUSIONS: Non-modifiable patient-related factors have a profound effect on early survival following a hybrid procedure for SVSOO. Outcomes for patients under 2.0 kg with aortic atresia remain very poor. Risk stratification should inform management decisions.

Hybrid palliation for critical systemic outflow obstruction: neither rapid stage 1 Norwood nor comprehensive stage 2 mitigate consequences of early risk factors.

OBJECTIVE: Hybrid palliation with branch pulmonary artery banding (bPAB) has become increasingly common in the early management of patients with critical left ventricular outflow obstruction. Optimal subsequent surgical palliation remains undefined. METHODS: We retrospectively reviewed patients undergoing initial bPAB for single ventricle physiology with systemic outflow obstruction (2001-2013, n = 37). Patients were stratified by subsequent surgical palliation: stage 1 Norwood (St1N, n = 14), comprehensive stage 2 (CompSt2, n = 11), and none (n = 12). RESULTS: bPAB was performed at a median of 4 days and 2.7-kg, post-bPAB mortality was increased in patients with aortic atresia (odds ratio [OR] = 3.8, 95% confidence interval [CI] = 0.9-15.8) or birth weight <2 kg (OR = 13.8, 95% CI = 1.4-136.4). Palliation strategy did not affect transplant-free survival through second-stage palliation (St1N: 71.4%, CompSt2: 72.7%, P = .9). Among CompSt2 patients, there was a trend toward poorer survival with aortic atresia (0% vs 80%, P = .09); birth weight <2.5 kg was associated with decreased survival (0% vs 89.0%, P = .01). A trend toward lower survival with low birth weight was evident among St1N patients (<2 kg, OR = 0.1, 95% CI = 0.01-1.9, P = .09). CompSt2 mortality occurred on postoperative days 0 and 1. Mortality following St1N occurred at a median of 38.5 days (range = 23.5-104.5). Among survivors of stage 2 palliation, Fontan completion was performed in the same number of patients in each group (St1N: 6/8, 75%, CompSt2: 6/8, 75%). CONCLUSIONS: Both St1N and CompSt2 are viable options for subsequent palliation following initial hybrid procedure. Transplant-free survival and eventual Fontan candidacy are similar between groups. Delaying surgical palliation with the CompSt2 did not mitigate the impact of early risk factors such as low birth weight and aortic atresia.

Acquired discrete subaortic stenosis in solid organ transplant recipients.

DSS is a rare acquired abnormality of the LV outflow tract in children and adults. DSS can be seen with or without associated congenital heart disease, but the mechanism of its emergence has been poorly understood. Here, we report two cases of DSS that developed after solid organ transplant in childhood. One patient underwent liver and kidney transplant at three yr of age for hereditary polycystic disease, and the other had liver transplant at three months of age due to uncontrollable high-output failure secondary to a large hepatic hemangioma. Both patients developed mild dynamic LV outflow tract obstruction and systemic hypertension soon after transplant, both of which resolved by the time of discharge. Transient LV hypertrophy was observed in both cases. Corticosteroids, cyclosporine A, and tacrolimus were used as initial immunosuppressants. Both patients developed DSS long after the regression of the initial dynamic LV outflow tract obstruction. Herein, we report DSS as an atypical late cardiovascular complication after solid organ transplant and postulate that transient post-transplant hyperdynamic LV outflow tract obstruction and chronic immunosuppressive treatment may lead to subendocardial remodeling resulting in a fibromuscular membranous ridge in susceptible subjects.

Device closure of secundum atrial septal defects in infants weighing less than 8 kg.

This study aimed to assess the technical aspects of atrial septal defect (ASD) closure using the Amplatzer septal occluder (ASO) and the Gore Helex septal occluder (GHSO) for infants weighing less than 8 kg and to determine the safety, effectiveness, and near-to-intermediate-term outcome of the closure. The Mid-Atlantic Group of Interventional Cardiology Registry of percutaneous, transcatheter ASD closure procedures was reviewed for this analysis. Patients from 10 hospitals in the United States were included. The cohort for this report consisted of 68 patients weighing less than 8 kg (range, 2.3-7.8 kg; mean, 5.5 ± 1.6 kg) and ranging in age from 1 to 24 months (mean, 8.6 ± 4.7 months). The indications for ASD closure were failure to thrive, significant right heart enlargement, shunts otherwise thought to be hemodynamically significant, and poor overall clinical status. Devices were successfully implanted in 66 of the 68 infants (97.1 % procedural success rate). Five minor procedure-related complications occurred. At follow-up assessment, clinical status had improved significantly as measured by improved weight gain and decreased ventilator or oxygen dependence. All residual shunts spontaneously closed during the follow-up period. Six late deaths occurred, none of which were clearly device related. The ASO and GHSO can be safely and effectively implanted for ASD closure in infants weighing less than 8 kg. These procedures usually are successful and seldom complicated, resulting in significant clinical improvement.

Bilateral pulmonary arterial banding results in an increased need for subsequent pulmonary artery interventions.

OBJECTIVE: Despite increasing use of bilateral branch pulmonary artery banding (bPAB), both as a temporary stabilizing treatment and as part of comprehensive hybrid management of hypoplastic left heart syndrome, little is known about the long-term outcomes of the pulmonary arteries (PAs) in banded patients. PATIENTS AND METHODS: We conducted a retrospective review of all patients with ductal-dependent systemic circulation (2001-2013) undergoing bPAB placement at a single institution (bPAB, n = 50); patients who underwent a stage I Norwood procedure (Norwood, n = 53) were used for comparison. The need for PA interventions (surgical arterioplasty, balloon angioplasty, and stent implantation) and PA growth were assessed. RESULTS: Bands were in place for a median of 76 days. PA growth and size were similar between groups, but bPAB patients required more interventions (1.4 ± 2.9 vs 0.5 ± 1.2, P = .01). In competing risks analysis, only 20% of bPAB patients were alive and free from intervention at 5 years after bPAB removal. Multivariable Cox proportional hazards regression of operative interventions within the bPAB group demonstrated the following risk factors: subsequent 2-ventricle repairs (hazard ratio [HR], 2.2; 95% confidence interval [CI], 0.7-6.7), smallest band diameter (HR per additional millimeter, 0.059; 95% CI, 0.004-0.849), and duration of band placement more than 90 days (HR, 3.5; 95% CI, 1.0-12.6). Hemodynamics and Fontan candidacy did not differ between groups. CONCLUSIONS: Patients with bPAB require additional interventions at earlier time points than Norwood patients. Patients with smaller bands and longer duration of banding are at high risk. Despite stenoses requiring additional interventions, Fontan candidacy is maintained.

Impact of pre-stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial.

OBJECTIVE: To compare the interstage cardiac catheterization hemodynamic and angiographic findings between shunt types for the Pediatric Heart Network Single Ventricle Reconstruction trial. The trial, which randomized subjects to a modified Blalock-Taussig shunt (MBTS) or right ventricle-to-pulmonary artery shunt (RVPAS) for the Norwood procedure, demonstrated the RVPAS was associated with a smaller pulmonary artery diameter but superior 12-month transplant-free survival. METHODS: We analyzed the pre-stage II catheterization data for the trial subjects. The hemodynamic variables and shunt and pulmonary angiographic data were compared between shunt types; their association with 12-month transplant-free survival was also evaluated. RESULTS: Of 549 randomized subjects, 389 underwent pre-stage II catheterization. A smaller size, lower aortic and superior vena cava saturation, and higher ventricular end-diastolic pressure were associated with worse 12-month transplant-free survival. The MBTS group had a lower coronary perfusion pressure (27 vs 32 mm Hg; P<.001) and greater pulmonary blood flow/systemic blood flow ratio (1.1 vs 1.0, P=.009). A greater pulmonary blood flow/systemic blood flow ratio increased the risk of death or transplantation only in the RVPAS group (P=.01). The MBTS group had fewer shunt (14% vs 28%, P=.004) and severe left pulmonary artery (0.7% vs 9.2%, P=.003) stenoses, larger mid-main branch pulmonary artery diameters, and greater Nakata indexes (164 vs 134, P<.001). CONCLUSIONS: Compared with the RVPAS subjects, the MBTS subjects had more hemodynamic abnormalities related to shunt physiology, and the RVPAS subjects had more shunt or pulmonary obstruction of a severe degree and inferior pulmonary artery growth at pre-stage II catheterization. A lower body surface area, greater ventricular end-diastolic pressure, and lower superior vena cava saturation were associated with worse 12-month transplant-free survival.

Intervention for recoarctation in the single ventricle reconstruction trial: incidence, risk, and outcomes.

BACKGROUND: Recoarctation after the Norwood procedure increases risk for mortality. The Single Ventricle Reconstruction (SVR) trial randomized subjects with a single right ventricle undergoing a Norwood procedure to a modified Blalock-Taussig shunt or a right ventricle-pulmonary artery shunt. We sought to determine the incidence of recoarctation, risk factors, and outcomes in the SVR trial. METHODS AND RESULTS: Recoarctation was defined by intervention, either catheter based or surgical. Univariate analysis and multivariable Cox proportional hazard models were performed with adjustment for center. Of the 549 SVR subjects, 97 (18%) underwent 131 interventions (92 balloon aortoplasty, 39 surgical) for recoarctation at a median age of 4.9 months (range, 1.1-10.5 months). Intervention typically occurred at pre-stage II catheterization (n=71, 54%) or at stage II surgery (n=38, 29%). In multivariable analysis, recoarctation was associated with the shunt type in place at the end of the Norwood procedure (hazard ratio, 2.0 for right ventricle-pulmonary artery shunt versus modified Blalock-Taussig shunt; P=0.02), and Norwood discharge peak echo-Doppler arch gradient (hazard ratio, 1.07 per 1 mm Hg; P<0.01). Subjects with recoarctation demonstrated comorbidities at pre-stage II evaluation, including higher pulmonary arterial pressures (15.4±3.0 versus 14.5±3.5 mm Hg; P=0.05), higher pulmonary vascular resistance (2.6±1.6 versus 2.0±1.0 Wood units·m(2); P=0.04), and increased echocardiographic volumes (end-diastolic volume, 126±39 versus 112±33 mL/BSA(1.3), where BSA is body surface area; P=0.02). There was no difference in 12-month postrandomization transplantation-free survival between those with and without recoarctation (P=0.14). CONCLUSIONS: Recoarctation is common after Norwood and contributes to pre-stage II comorbidities. Although with intervention there is no associated increase in 1-year transplantation/mortality, further evaluation is warranted to evaluate the effects of associated morbidities.

Complex atrial septal defect: percutaneous repair guided by three-dimensional echocardiography.

Three-dimensional transesophageal echocardiography (3D TEE) has been used to guide the percutaneous repair of simple atrial septal defects (ASDs). There has been limited experience in using this imaging modality to guide complex ASD repair. In this report, we describe how 3D TEE was used to guide the repair of a complex, multifenestrated ASD. In a single view, 3D TEE provides a superior anatomic definition when compared to the traditional two-dimensional echocardiography. We believe that this emerging technology will play a critical role as the number and complexity of percutaneous techniques treating structural heart disease continue to rise. (Echocardiography 2010;27:590-593).