Raghav Murthy

Utilization of the impella 2.5 micro-axial pump in children for acute circulatory support

Percutaneous circulatory support is widely available for use in adult patients. Options remain limited in the pediatric population due to limitations of vessel and patient size, resulting in the use of more invasive means of circulatory support. We present a case series of pediatric patients in whom the Impella 2.5 axial flow pump was able to be placed utilizing a hybrid approach that allowed for adequate distal perfusion while providing successful short‐term hemodynamic support.

Early Outcomes With HeartWare HVAD as Bridge to Transplant in Children: A Single Institution Experience

The HeartWare HVAD has been used as a bridge to cardiac transplantation in the pediatric population. We describe outcomes following HeartWare HVAD implantation at a single center. A retrospective chart review was performed of all HeartWare HVAD implants performed at our institution between May 2013 and March 2015. Eight children between the ages of 9 and 17 years underwent HVAD implantation as a bridge to transplant (N = 7 cardiomyopathy, N = 1 complex single ventricle). There was one operative death in the complex single ventricle patient. Seven patients (87%) were successfully bridged to transplant. Median time of support was 24.5 days (range, 6-91 days). All transplanted patients are alive and well at a median follow-up of 448 days. Our results demonstrated that mechanical support with HeartWare HVAD is feasible in patients of varying sizes (from older children to adolescents).

Heart Xenotransplantation: Historical Background, Experimental Progress, and Clinical Prospects

If pig hearts could be transplanted successfully into patients with end-stage cardiac failure, the critical shortage of hearts from deceased human donors would be overcome. The several attempts at cardiac xenotransplantation carried out in the 20th century, usually with hearts from nonhuman primates (NHPs), are reviewed, as are the surgical techniques used in experimental heart transplantation in animals. For a number of reasons, the pig has been selected as the potential source of organs for clinical transplantation. The major pathobiological barriers that the pig presents, and progress in overcoming these barriers either by genetic engineering of the pig or by the administration of novel immunosuppressive agents, are described. Currently, non-life-supporting pig heterotopic heart transplantation in NHPs has extended to more than 2 years in 1 case, with life-supporting orthotopic heart transplantation of almost 2 months. Future approaches to resolve the remaining problems and the selection of patients for the initial clinical trials are briefly discussed.

Management and Outcomes of Heterotaxy Syndrome Associated With Pulmonary Atresia or Pulmonary Stenosis

BACKGROUND Historic outcomes of patients with heterotaxy and pulmonary atresia or pulmonary stenosis (PA/PS) have been poor and in the current era are incompletely described. We reviewed our management of these patients and associated risk factors for death. METHODS We retrospectively reviewed the records of all patients with heterotaxy and PA/PS treated in our institution from January 1, 2002, to August 31, 2012. Death data were also confirmed with the Social Security Death Index. The log-rank test was done to assess six risk factors for death. RESULTS We identified 42 patients with heterotaxy and PA/PS. Median age at the first operation was 6.5 days, and median follow-up was 3.5 years. Death data were complete for all patients. Overall mortality was 19% (8 of 42). The 30-day, 1-year, and 5-year mortality estimation was 4.76%, 12.3%, and 19.1% respectively, as determined by the Kaplan-Meier method. The log-rank test showed total anomalous pulmonary venous return (TAPVR) (p<0.05) and obstructed TAPVR requiring an operation at less than 30 days (p=0.001) were significant risk factors for death. CONCLUSIONS In the current era, surgical treatment of heterotaxy and PA/PS can result in good outcomes. Associated TAPVR and obstructed TAPVR requiring neonatal correction were noted to be risk factors for death.

Selective Use of the Blalock-Taussig Shunt and Right Ventricle-to-Pulmonary Artery Conduit During the Norwood Procedure

Background: The single ventricle reconstruction trial showed better one-year transplant-free survival for the right ventricle-to-pulmonary artery (RV-to-PA) conduit over the modified Blalock-Taussig shunt (mBTS) at Norwood operation. However, concerns remain about the long-term effects of a neonatal ventriculotomy. In our institution, we have used specific selection criteria for the use of mBTS in the Norwood operation. Methods: We reviewed 122 consecutive neonates undergoing the Norwood procedure from December 2006 to December 2013. We used the following criteria to select our source of pulmonary blood flow: (1) presence of a dominant morphologic left ventricle; (2) presence of antegrade blood in an ascending aorta that is greater than 3 mm; and (3) presence of significant large “crossing coronaries” on ventricle. All patients who met any of the above 3 criteria underwent an mBTS while the remaining patients underwent an RV-to-PA conduit. Results: Seventy-five (61.5%) patients had the RV-to-PA conduit and 47 (38.5%) patients had an mBTS. The overall surgical mortality was 9%. Mean follow-up interval was 23.5 months. Actuarial transplant-free survival was similar at 12, 24, 36, and 48 months in both the mBTS group and the RV-to-PA conduit group. In the RV-to-PA conduit group, actuarial transplant-free survival was 73% at 12 months, 71% at 24 months, 71% at 36 months, and 67% at 48 months, while in the mBTS group, actuarial transplant-free survival was 82% at 12 months, 75% at 24 months, 75% at 36 months, and 75% at 48 months. Conclusion: Our selection criteria for mBTS have allowed us to obtain equivalent transplant-free survival at 12, 24, 36, and 48 months when compared to the RV-to-PA conduit group.

Coxiella burnetti‐associated thoracic endovascular stent graft infection

A 69-year-old female underwent a thoracic endovascular aneurysm repair (TEVAR) for a 7 × 5.4-cm Crawford type 1 thoracoabdominal aneurysm. She was subsequently readmitted with fatigue, weight loss, night sweats, and abdominal pain. A computed tomogram angiogram (CTA) demonstrated an enlarging aneurysmal mass with thickened walls concerning for an infected TEVAR graft (Fig. 1). An extensive workup revealed an increased IgG titer; phase I 1:16384, phase II 1:8182 consistent with a Coxiella burnetti infection. Doxycyline (200mg/day) and hydroxychloroquine (200mg tid) were instituted to decrease titers to <1:800, to minimize the risk of aerosolization and potential exposure to healthcare personnel. During this period of medical therapy, she continued to have fatigue and weight loss and developed hematemesis. A follow-up CTA now demonstrated perianeurysmal air and fat stranding concerning for an aorto-graftesophageal fistula (Fig. 2). She underwent emergent surgical repair through a left posterolateral thoracoabdominal incision using left heart bypass instituted with cannulation of the transverse arch and an 8-mm Hemashield graft (MAQUET Holding B.V. & Co. KG, Rastatt, Germany)

Aortoesophageal Fistula in a Child With Undiagnosed Vascular Ring: Life-Threatening or Lethal?

Exsanguinating hematemesis secondary to an aortoesophageal fistula from an impacted foreign body occurred in a patient with a vascular ring. This report describes successful resuscitation and repair in a 6-year-old boy who was transferred from an outside hospital in extremis with an aortoesophageal fistula from a foreign body in the presence of a vascular ring.

Single-Center Experience With the Senning Procedure in the Current Era

The Senning procedure is an operative technique for atrial inversion in congenital heart anomalies. We sought to evaluate our contemporary outcomes employing this technique. A retrospective analysis of all patients who underwent the Senning procedure at our institution was performed. Hospital records were reviewed, and follow-up data were obtained to evaluate outcomes. Overall, a total of 19 patients underwent a Senning procedure between August 2005 and July 2014. Median age at repair was 594 days (range: 5 days to 15 years). Of those, 7 patients underwent a combined Senning-Rastelli operation and 10 patients underwent a double switch operation (arterial switch or atrial switch). Primary diagnosis was congenitally corrected transposition of the great arteries (cc-TGA) in 17 patients. Associated lesions included ventricular septal defect (n = 10), pulmonary stenosis or atresia (n = 7), and Ebstein׳s anomaly of the tricuspid valve (n = 4). There was an operative death (5%). In all, 2 patients required superior vena cava baffle revision in the operating room. No new pulmonary venous or systemic venous baffle obstruction was observed during follow-up. A total of 5 (26%) patients developed heart block requiring pacemaker implantation. Actuarial survival was 81% at 5 years with a median follow-up of 38 months. The 2 patients in the cc-TGA group were subsequently transplanted. Surviving patients (n = 15) are all well at the last clinical follow-up, most with normal biventricular function. In conclusion, the Senning procedure offers excellent outcomes with regard to systemic and pulmonary venous baffle patency. Results in patients with cc-TGA, however, are affected by significant early mortality after Senning-Rastelli and incidence of left ventricular dysfunction after double switch; which should be considered during patient selection and surgical planning.

Arterial Switch Operation in a Patient With Ehlers-Danlos Syndrome Type IV

Ehlers-Danlos syndrome vascular type IV is characterized by translucent skin, easy bruising, and fragility of arteries. A full-term female infant presented at four weeks of age with a diagnosis of d-transposition of the great arteries with restrictive atrial septal defect. She successfully underwent emergent balloon atrial septostomy and placement of patent ductus arteriosus (PDA) stent. She required restenting of the PDA and pulmonary artery banding prior to arterial switch procedure. At 16 months of age, the patient successfully underwent arterial switch procedure without complication. This report demonstrates the feasibility of an arterial switch operation along with long-term follow-up of this rare condition.

Epicardial Intraoperative Three-Dimensional Mapping of Wolff-Parkinson-White Syndrome in a Child With Ebstein’s Anomaly

Catheter ablation of arrhythmias in small patients has inherent significant risk because of the patient, catheter, and lesion size. Heart block, perforation, and coronary damage may occur. This report shows adaptation of a three-dimensional electroanatomic mapping system for intraoperative use in a small patient with Ebsteins anomaly who was undergoing a cone procedure and a bidirectional Glenn procedure. Use of this technique gave clear identification of the epicardial insertion points of two accessory pathways causing Wolff-Parkinson-White syndrome and resistant episodes of supraventricular tachycardia. Successful three-dimensional mapping and cryoablation were performed with a short procedure time and no complications. The patient remains without preexcitation or arrhythmia 6 months postoperatively.