Sarosh P. Batlivala

Pulmonary Valve Replacement Function in Adolescents: A Comparison of Bioprosthetic Valves and Homograft Conduits

BACKGROUND Data comparing function and reintervention rates of various pulmonary valve replacements in adolescents are sparse. New transcatheter therapies-including transcatheter pulmonary valves-have been developed, and their utilization could be justified by these data. METHODS We performed a retrospective review that included baseline and operative data with cross-sectional follow-up on adolescents aged 10 to 21 years who underwent pulmonary valve replacement (n=254). We compared homograft conduits (n=84) with bioprosthetic pulmonary valves (n=170) in terms of freedom from valve dysfunction--right ventricular outflow tract obstruction greater than 50 mm Hg, or moderate or greater pulmonary regurgitation--and reintervention. RESULTS Median follow-up duration was 4.4 years and was longer in the homograft cohort. Freedom from valvar dysfunction was 72%±4% at 5 years and 48%±8% at 10 years. Freedom from right ventricular outflow tract reintervention was 90%±3% at 5 years, 67%±5% at 10 years, and 47%±8% at 15 years. No differences existed for dysfunction or reintervention between the homograft and bioprosthetic valve cohorts. Shorter freedom from dysfunction was associated with younger age, smaller implanted valve Z-score, valve replacement after prior homograft, and higher preoperative right ventricular outflow tract (RVOT) gradient. Shorter freedom from reintervention was associated with younger age, smaller implanted valve Z-score, and more recent valve implant. CONCLUSIONS Adolescents represent an important age group as an increasing number are undergoing pulmonary valve replacement, and they may be particularly impacted by new transcatheter therapies. This study provides baseline comparative data for current surgical options that should help inform medical decision making as longer term data become available for transcatheter pulmonary valve replacement and other new technologies.

“How to Do It” Hybrid Stent Placement for Pulmonary Vein Stenosis

Pulmonary vein stenosis (PVS) is often progressive and severe. Surgical and percutaneous angioplasty are acutely successful; however, restenosis is common and many patients require multiple reinterventions. We perform intraoperative “hybrid” stent placement to deliver larger, stronger stents. Hybrid stent placement is well described for pulmonary arterial stenosis (PAS). The PAS data demonstrate that smaller stents are associated with rapid in-stent restenosis. Data from PVS in adults demonstrate superior outcomes with larger stents. Hybrid stent placement requires a strong collaborative effort between congenital heart surgeons and interventional cardiologists.

Isolated pulmonary artery arising from a duct: A single-center review of diagnostic and therapeutic strategies

OBJECTIVE Isolated pulmonary artery of ductal origin (IPADO) is a rare disease with diverse presentations. Diagnostic and therapeutic approaches vary widely given the low incidence. Reporting additional cases may help inform medical decision making. METHODS We reviewed diagnostic data--including echocardiography, catheterization, and MRI--and outcomes for all patients with IPADO at our institution. RESULTS Thirty-seven patients who met inclusion criteria were identified. The mean age at diagnosis was 3 months (range, 1 day-45 years). In 11 patients, the duct supplying the IPADO was patent (ie, patent ductus arteriosis [PDA]); the duct had closed (ligamentum) in the other 26 patients. When performed, catheterization delineated the anatomy in 90% (100% if PDA) versus 54% with magnetic resonance imaging. Patients with a PDA were more likely to undergo intervention (100% vs 58%, P=.02) and had earlier first intervention (1 vs 20 months; P<.001). Patients diagnosed at age≤6 months were more likely to undergo intervention (86% vs 50%; P=.03) and unifocalization (81% vs 44%; P=.04), and had greater IPADO flow at follow-up (40% vs 14%; P<.001). Patients who underwent any intervention had greater IPADO flow than those without intervention (38% vs 0%). CONCLUSIONS Early IPADO diagnosis is important in long-term outcome. However, successful interventions can be performed on older patients. Diagnosis relies on angiography but magnetic resonance imaging may play an increasingly important role. Although initial intervention depends on individual factors, the ultimate goal should be early unifocalization.

Percutaneous Closure of Perimembranous Ventricular Septal Defects Using the Second-Generation Amplatzer Vascular Occluders.

Earlier attempts at percutaneous closure of perimembranous ventricular septal defects (Pm VSDs) were abandoned because of incidence of heart block likely as a result of device rigidity and/or oversizing. This is retrospective review and data reporting of patients who underwent percutaneous closure using the softer second-generation Amplatzer vascular occluders; namely the Amplatzer vascular plug, second generation, (AVP II) and the Amplatzer duct occluder, second generation (ADO II) in our institution. A total of 20 patients were identified; AVP II was used in 9 patients and ADO II in 11 patients. Median weight was 13.45 kg (range 6.5 to 76); age 28.5 months (range 11 to 352). After procedure, 4 were noted to have aortic insufficiency; trivial in 3 and mild in 1 (unrelated to the device). Mild tricuspid regurgitation possibly device or procedure related was seen in 4. Residual flow through the device was common after procedure and disappeared in all but 3, graded as trivial in 1, small in 2. Average follow-up period was 7.54 months ± 7.5 (1 day to 25 months). There was no incidence of heart block, bacterial endocarditis, hemolysis, device embolization, or fracture. The aortic insufficiency resolved in 1 patient and was estimated to be trivial in the remaining 3 patients. In conclusion, percutaneous closure of Pm VSDs using the softer new generation devices as the AVP II and the ADO II is feasible and safe. Longer follow-up and larger series are needed.

Achieving Benchmark Results for Neonatal Palliation of Hypoplastic Left Heart Syndrome and Related Anomalies in an Emerging Program

Background: Results of surgical management of hypoplastic left heart syndrome (HLHS) and related anomalies are often compared to published benchmark data which reflect the use of a variety of surgical and hybrid protocols. We report encouraging results achieved in an emerging program, despite a learning curve at all care levels. Rather than relying on a single preferred protocol, surgical management was based on matching surgical strategy to individual patient factors. Methods: From 2010 to 2014, a total of 47 consecutive patients with HLHS or related anomalies with ductal-dependent systemic circulation underwent initial surgical palliation, including 30 Norwood stage I, 8 hybrid stage I, and 9 salvage-to-Norwood procedures. True hybrid procedures entailed bilateral pulmonary artery banding and ductal stenting. In the salvage-to-Norwood strategy, ductal stenting was withheld in favor of continued prostaglandin infusion in anticipation of a deferred Norwood procedure. Cardiac comorbidities (obstructed pulmonary venous return, poor ventricular function, and atrioventricular valve regurgitation) and noncardiac comorbidities influenced the choice of treatment strategies and were analyzed as potential risk factors for extracorporeal membrane oxygenation (ECMO) support or in-hospital mortality. Results: Overall hospital survival was 81% (Norwood 83.3%, hybrid 88%, “salvage” 67%; P = .4942). Extracorporeal membrane oxygenation support was used for eight (17%) patients with two survivors. For cases with obstructed pulmonary venous return (n = 10, 21%), management choices favored a hybrid or salvage strategy (P = .0026). Aortic atresia (n = 22, 47%) was treated by a Norwood or salvage-to-Norwood. No cardiac, noncardiac, or genetic comorbidities were identified as independent risk factors for ECMO or discharge mortality in a multivariable analysis. Conclusions: Our emerging program achieved outcomes that compare favorably to published benchmark data with respect to hospital survival. These results reflect rigorous interdisciplinary teamwork and a flexible approach to surgical palliation based on matching surgical strategy to patient factors. With major associated cardiac/noncardiac comorbidity and antegrade coronary flow, a true hybrid with ductal stenting was our preferred strategy. For high-risk situations such as aortic atresia with obstructed pulmonary venous return, the salvage hybrid-bridge-to-Norwood strategy may help achieve survival albeit with increased resource utilization.

Unusual cause of aborted sudden cardiac death in a teen athlete: homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Common aetiologies of sudden cardiac death in children include coronary anomalies, channelopathies, and cardiomyopathies. Less frequently, hypercoagulable states cause sudden arrest. We report an unusual case of aborted sudden cardiac death in a teenager, ultimately found to have homozygosity for the 4G allele of the plasminogen activase inhibitor type 1 gene.

Staged Palliation of Cyanotic Obstructive Lesions With a Modified Right Ventricular Outflow Procedure

Background: Traditional palliation for biventricular cyanotic congenital heart lesions often involves staging with systemic-to-pulmonary arterial shunts to secure pulmonary blood flow (PBF) in the newborn period prior to complete repair. However, shunts may lead to life-threatening events secondary to shunt occlusion or acute coronary steal. They may be associated with morbidity secondary to diastolic runoff, systemic steal and volume loading, and do not provide pulsatile flow which has the potential to promote pulmonary artery (PA) growth. We have alternatively performed modified right ventricular outflow (mRVO) procedures by establishing antegrade right ventricle-to-PA flow. Methods: Retrospective review of data on all patients who underwent the mRVO procedure from 2013 to 2016, including anatomy, number of interstage catheterizations, reoperations, intensive care unit admissions, hypercyanotic episodes, interval to complete repair, and mortality. Results: Seventeen nonconsecutive patients included tetralogy of Fallot (n = 14), pulmonary valve stenosis (n = 2), and 1 with pulmonary atresia-intact septum; 14 had significant branch PA stenosis. Median age of first mRVO procedure was 14 days (range 5-193), and median duration of follow-up was 15.3 months (range 4-47 months). No patients had post-palliation acute hypercyanotic episodes. Nine were admitted to the ICU for persistent interstage hypoxemia, 7 of whom required reintervention prior to complete repair, which was achieved in 11 patients. Two late deaths unrelated to mRVO occurred. Conclusions: The mRVO procedure is a potential option with satisfactory results. It avoids potential shunt-related sudden death. The physiology of the mRVO palliation may provide unique benefits by providing antegrade pulsatile PBF, facilitates catheter interventions, and avoids branch PA distortion and stenosis.

Isolated Left Subclavian Artery From the Pulmonary Artery Masked by Pulmonary Hypertension.

Isolated subclavian artery arising from the pulmonary artery is exceedingly rare. Most cases are associated with other forms of congenital heart disease and typically present with subclavian or pulmonary steal syndromes. We report the case of a patient with pulmonary hypertension which masked the isolated subclavian artery, allowing it to masquerade as another common congenital heart lesion.

Not-so-identical twins with trisomy 21 and perimembranous ventricular septal defects.

While trisomy 21 is a common genetic disorder in singletons, the incidence among identical twins is very rare, occurring in approximately 1-2 per 1000 twin gestations. Trisomy 21 is associated with high incidence of congenital heart defects, and commonly occurs with ventricular septal defects (VSDs). Physiologic burden of VSDs depends on prevalence of anatomic and other circulatory factors. A case of identical twins with trisomy 21 and large VSDs is described in the present report. Though genetically identical, phenotypes varied significantly. One twin was managed medically, while the other developed more significant heart failure, requiring operative repair.

Hypoplastic left heart syndrome and ventriculocoronary connections.

Hypoplastic left heart syndrome (HLHS) is a critical congenital heart defect. There are variants of HLHS, consisting of permutations of stenosis or atresia of the mitral and aortic valves. A notable phenomenon described in all HLHS subtypes is ventriculocoronary connections (VCCs). The significance of VCCs remains undetermined. Some studies have demonstrated higher mortality associated with VCCs, while others have shown no difference in mortality compared to other HLHS variants. All variants of HLHS are associated with myocardial disarray and endomyocardial fibroelastosis, although recent evidence demonstrates a higher association of those findings with the mitral stenosis–aortic atresia (MS-AA) variant. And VCCs may have a higher incidence in MS-AA as they constitute the only egress of blood from the LV. These associations may play a role in the survival of patients with MS-AA. We present a case of HLHS with MS-AA and significant VCCs. Figure 1 depicts the diminutive LV, no outflow, and opacification of the entire coronary system. Coronary opacification can only be achieved via VCCs, given the aortic valve atresia. Figure 2 demonstrates the severely hypoplastic ascending aorta. The aorta is similar in size to the 3F catheter and the coronaries do not opacify.