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Successful Heart Transplantation in Two Infants With Dextrocardia-Heterotaxy Syndrome

Boston, Umar; Kumar, Tk Susheel; Shah, Jignesh; Street, Tiffany; Towbin, Jeffrey; Knott-Craig, Christopher J
Infants with dextrocardia-heterotaxy syndrome pose significant surgical challenges to a successful orthotopic heart transplant. Herein we report on 2 infants with dextrocardia-heterotaxy syndrome who underwent complex heart transplantation. A combination of preoperative 3-dimensional chest computed tomography to predict optimal donor size and unique surgical technique resulted in successful outcomes.
PMID: 30081023
ISSN: 1552-6259
CID: 3664612

Biventricular root replacement for transcatheter pulmonary valve endocarditis after Ross operation: Double the trouble

Kumar, T K Susheel; Balduf, Kaitlin; Boston, Umar; Knott-Craig, Christopher
PMID: 30391009
ISSN: 1097-685x
CID: 3664602

Surgical management of patent ductus arteriosus

Susheel Kumar, Thittamaranahalli Kariyappa
Surgical ligation of patent ductus arteriosus can be performed safely by following standard steps of operation. Familiarity of the anatomical landmarks and simple precautions result in high degree of safety. The technique of surgical ligation varies with the age of the patient. Surgical ligation of ductus is associated with well-recognized complications, although they occur only in a small number of patients.
PMID: 30811804
ISSN: 1747-0803
CID: 4418472

Management of coronary artery arising from nonfacing sinus in transposition of great arteries

Kumar, T K Susheel; Amin, Nomisha; Sathanandam, Shyam; Knott-Craig, Christopher J
PMID: 29935794
ISSN: 1097-685x
CID: 3664572

Surgical Management and Outcomes of Ebstein Anomaly in Neonates and Infants: A Society of Thoracic Surgeons Congenital Heart Surgery Database Analysis

Holst, Kimberly A; Dearani, Joseph A; Said, Sameh M; Davies, Ryan R; Pizarro, Christian; Knott-Craig, Christopher; Kumar, T K Susheel; Starnes, Vaughn A; Kumar, S Ram; Pasquali, Sara K; Thibault, Dylan P; Meza, James M; Hill, Kevin D; Chiswell, Karen; Jacobs, Jeffrey P; Jacobs, Marshall L
BACKGROUND:Ebstein anomaly (EA) encompasses a broad spectrum of morphology and clinical presentation. Those who are symptomatic early in infancy are generally at highest risk, but there are limited data regarding multicentric practice patterns and outcomes. We analyzed multiinstitutional data concerning operations and outcomes in neonates and infants with EA. METHODS:Index operations reported in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) were potentially eligible for inclusion. Analysis was limited to patients with diagnosis of EA and less than 1 year of age at time of surgery (neonates ≤30 days, infants 31 to 365 days). RESULTS:The study population included 255 neonates and 239 infants (at 95 centers). Among neonates, median age at operation was 7 days (interquartile range, 4 to 13 days) and the majority required preoperative ventilation (61.6%, n = 157). The most common primary operation performed among neonates was Ebstein repair (39.6%, n = 101), followed by systemic-to-pulmonary shunt (20.4%, n = 52) and tricuspid valve closure (9.4%, n = 24). Overall neonatal operative mortality was 27.4% (n = 70), with composite morbidity-mortality of 51.4% (n = 48). For infants, median age at operation was 179 days (interquartile range, 108-234 days); the most common primary operation for infants was superior cavopulmonary anastomosis (38.1%, n = 91) followed by Ebstein repair (15.5%, n = 37). Overall operative mortality for infants was 9.2% (n = 22) with composite morbidity-mortality of 20.1% (48). CONCLUSIONS:Symptomatic EA in early infancy is very high risk and a variety of operative procedures were performed. A dedicated prospective study is required to more fully understand optimal selection of treatment pathways to guide a systematic approach to operative management.
PMID: 29777671
ISSN: 1552-6259
CID: 3664562

Double aortic arch presenting as neonatal respiratory failure in the delivery room [Case Report]

Lee, Marc; Kumar, T K Susheel; Paudel, Govinda
Respiratory symptoms are prevalent in vascular ring anomalies, including double aortic arch, which is considered the most common type of vascular ring. However, respiratory failure owing to critical airway obstruction in the neonatal period is rare with few reported cases in the literature. We report a case of a newborn patient who required emergent intubation within the first few hours of life and was later discovered to have a double aortic arch.
PMID: 29248031
ISSN: 1467-1107
CID: 3664522

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

Briceno-Medina, Mario; Kumar, T K Susheel; Sathanandam, Shyam; Boston, Umar; Perez, Michael; Allen, Jerry; Zurakowski, David; Ilbawi, Michel; Knott-Craig, Christopher J
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.
PMID: 28847337
ISSN: 1467-1107
CID: 3664472

Successful Use of Aortic Homograft for Residual Tracheal Stenosis Following Slide Tracheoplasty [Case Report]

Kumar, T K Susheel; Immanuel, Camille; Thompson, Jerome; Shah, Samir; Knott-Craig, Christopher
PMID: 29518535
ISSN: 1532-9488
CID: 3664542

Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach [Case Report]

Balduf, Kaitlin; Kumar, T K Susheel; Boston, Umar; Sathanandam, Shyam; Lee, Marc V; Jancelewicz, Tim; Knott-Craig, Christopher J
Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.
PMID: 29448010
ISSN: 1532-9488
CID: 3664532

Optimum position of transthoracic intracardiac line following cardiac surgery

Kumar, T K Susheel; Subramanian, Saradha; Tansey, James Benjamin; Figueroa, Mayte; Zurakowski, David; Allen, Jerry; Shah, Samir; Brauninger, Anna; Bird, Lindsey; Ali, Mohammed; Knott-Craig, Christopher J
OBJECTIVES:The transthoracic intracardiac line placed in the right atrium provides a convenient access to the central venous system following cardiac surgery. However, it is associated with risks such as migration and bleeding. We conducted a retrospective study to determine whether position of transthoracic line with respect to site of exit from the chest makes a difference in the rate of complications. METHODS:All infants receiving a transthoracic intracardiac line in the right atrium following cardiac surgery between June 2012 and December 2015 were part of the study. A 3.5-Fr double-lumen umbilical venous catheter was placed directly into the right atrium. The lines exited the thorax either above in the suprasternal notch (upper transthoracic line) or below the diaphragm across the abdominal wall (lower transthoracic line). Patients were analysed for complications such as catheter migration, bleeding upon removal, atrial thrombus, line occlusion, premature removal and failed removal. RESULTS:A total of 131 patients received a transthoracic intracardiac line during the study period. Of the total patients, 88 patients received the upper transthoracic line and 43 patients received the lower transthoracic line. The upper transthoracic line was associated with significantly lower incidence of catheter migration (1% vs 14%) and this held by multivariable logistic regression, adjusting for age and duration of the line (P = 0.003). There was no difference in the rate of other complications including bleeding. CONCLUSIONS:The upper transthoracic line is associated with significantly lower incidence of catheter migration and offers a more optimum position for central access following cardiac surgery.
PMID: 29106565
ISSN: 1569-9285
CID: 3664512