Prospective Study of TMVR Using Balloon-Expandable Aortic Transcatheter Valves in MAC: MITRAL Trial 1-Year Outcomes.

OBJECTIVES: The aim of this study was to evaluate 1-year outcomes of valve-in-mitral annular calcification (ViMAC) in the MITRAL (Mitral Implantation of Transcatheter Valves) trial. BACKGROUND: The MITRAL trial is the first prospective study evaluating the feasibility of ViMAC using balloon-expandable aortic transcatheter heart valves. METHODS: A multicenter prospective study was conducted, enrolling high-risk surgical patients with severe mitral annular calcification and symptomatic severe mitral valve dysfunction at 13 U.S. sites. RESULTS: Between February 2015 and December 2017, 31 patients were enrolled (median age 74.5 years [interquartile range (IQR): 71.3 to 81.0 years], 71% women, median Society of Thoracic Surgeons score 6.3% [IQR: 5.0% to 8.8%], 87.1% in New York Heart Association functional class III or IV). Access was transatrial (48.4%), transseptal (48.4%), or transapical (3.2%). Technical success was 74.2%. Left ventricular outflow tract obstruction (LVOTO) with hemodynamic compromise occurred in 3 patients (transatrial, n = 1; transseptal, n = 1; transapical, n = 1). After LVOTO occurred in the first 2 patients, pre-emptive alcohol septal ablation was implemented to decrease risk in high-risk patients. No intraprocedural deaths or conversions to open heart surgery occurred during the index procedures. All-cause mortality at 30 days was 16.7% (transatrial, 21.4%; transseptal, 6.7%; transapical, 100% [n = 1]; p = 0.33) and at 1 year was 34.5% (transatrial, 38.5%; transseptal, 26.7%; p = 0.69). At 1-year follow-up, 83.3% of patients were in New York Heart Association functional class I or II, the median mean mitral valve gradient was 6.1 mm Hg (IQR: 5.6 to 7.1 mm Hg), and all patients had ≤1+ mitral regurgitation. CONCLUSIONS: At 1 year, ViMAC was associated with symptom improvement and stable transcatheter heart valve performance. Pre-emptive alcohol septal ablation may prevent transcatheter mitral valve replacement-induced LVOTO in patients at risk. Thirty-day mortality of patients treated via transseptal access was lower than predicted by the Society of Thoracic Surgeons score. Further studies are needed to evaluate safety and efficacy of ViMAC.

Prospective Evaluation of Transseptal TMVR for Failed Surgical Bioprostheses: MITRAL Trial Valve-in-Valve Arm 1-Year Outcomes.

OBJECTIVES: The aim of this study was to assess 1-year clinical outcomes among high-risk patients with failed surgical mitral bioprostheses who underwent transseptal mitral valve-in-valve (MViV) with the SAPIEN 3 aortic transcatheter heart valve (THV) in the MITRAL (Mitral Implantation of Transcatheter Valves) trial. BACKGROUND: The MITRAL trial is the first prospective study evaluating transseptal MViV with the SAPIEN 3 aortic THV in high-risk patients with failed surgical mitral bioprostheses. METHODS: High-risk patients with symptomatic moderate to severe or severe mitral regurgitation (MR) or severe mitral stenosis due to failed surgical mitral bioprostheses were prospectively enrolled. The primary safety endpoint was technical success. The primary THV performance endpoint was absence of MR grade ≥2+ or mean mitral valve gradient ≥10 mm Hg (30 days and 1 year). Secondary endpoints included procedural success and all-cause mortality (30 days and 1 year). RESULTS: Thirty patients were enrolled between July 2016 and October 2017 (median age 77.5 years [interquartile range (IQR): 70.3 to 82.8 years], 63.3% women, median Society of Thoracic Surgeons score 9.4% [IQR: 5.8% to 12.0%], 80% in New York Heart Association functional class III or IV). The technical success rate was 100%. The primary performance endpoint in survivors was achieved in 96.6% (28 of 29) at 30 days and 82.8% (24 of 29) at 1 year. Thirty-day all-cause mortality was 3.3% and was unchanged at 1 year. The only death was due to airway obstruction after swallowing several pills simultaneously 29 days post-MViV. At 1-year follow-up, 89.3% of patients were in New York Heart Association functional class I or II, the median mean mitral valve gradient was 6.6 mm Hg (interquartile range: 5.5 to 8.9 mm Hg), and all patients had MR grade ≤1+. CONCLUSIONS: Transseptal MViV in high-risk patients was associated with 100% technical success, low procedural complication rates, and very low mortality at 1 year. The vast majority of patients experienced significant symptom alleviation, and THV performance remained stable at 1 year.

Prospective Evaluation of TMVR for Failed Surgical Annuloplasty Rings: MITRAL Trial Valve-in-Ring Arm 1-Year Outcomes.

OBJECTIVES: The authors report 1-year outcomes of high-risk patients with failed surgical annuloplasty rings undergoing transseptal mitral valve-in-ring (MViR) with the SAPIEN 3 aortic transcatheter heart valve (THV). BACKGROUND: The MITRAL (Mitral Implantation of Transcatheter Valves) trial is the first prospective study evaluating transseptal MViR with the SAPIEN 3 aortic THV in high-risk patients with failed surgical annuloplasty rings. METHODS: Prospective enrollment of high-risk patients with symptomatic moderate to severe or severe mitral regurgitation (MR) or severe mitral stenosis and failed annuloplasty rings at 13 U.S. sites. The primary safety endpoint was technical success. The primary THV performance endpoint was absence of MR grade ≥2+ or mean mitral valve gradient ≥10 mm Hg (30 days and 1 year). Secondary endpoints included procedural success and all-cause mortality (30 days and 1 year). RESULTS: Thirty patients were enrolled between January 2016 and October 2017 (median age 71.5 years [interquartile range: 67.0 to 76.8 years], 36.7% women, median Society of Thoracic Surgeons score 7.6% [interquartile range: 5.1% to 11.8%], 76.7% in New York Heart Association functional class III or IV). Technical success was 66.7% (driven primarily by need for a second valve in 6 patients). There was no intraprocedural mortality or conversion to surgery. The primary performance endpoint was achieved in 85.7% of survivors at 30 days (24 of 28) and 89.5% of patients alive at 1 year with echocardiographic data available (17 of 19). All-cause mortality at 30 days was 6.7% and at 1 year was 23.3%. Among survivors at 1-year follow-up, 84.2% were in New York Heart Association functional class I or II, the median mean mitral valve gradient was 6.0 mm Hg (interquartile range: 4.7 to 7.3 mm Hg), and all had ≤1+ MR. CONCLUSIONS: Transseptal MViR was associated with a 30-day mortality rate lower than predicted by the Society of Thoracic Surgeons score. At 1 year, transseptal MViR was associated with symptom improvement and stable THV performance.

Hybrid approach to postmyocardial infarction ventricular septal defect repair.

Postinfarction ventricular septal rupture is a rare and devastating complication of myocardial infarction. Despite attempts at acute surgical and percutaneous defect closure, morbidity and mortality remain high. Herein, we describe a hybrid surgical and catheter-based approach to defect closure in a 63-year-old woman with postinfarction ventricular septal rupture and cardiogenic shock.

Open atrial transcatheter mitral valve replacement in patients with mitral annular calcification.

BACKGROUND: Mitral valve replacement in the setting of severe mitral annular calcification remains a surgical challenge. Transcatheter mitral valve replacement (TMVR) using an aortic balloon-expandable transcatheter heart valve is emerging as a potential treatment option for high surgical risk patients. Transseptal, transapical, or transatrial access is not always feasible, so an understanding of alternative implantation techniques is important. OBJECTIVES: The authors sought to present a step-by-step description of a contemporary transatrial TMVR technique using balloon-expandable aortic transcatheter heart valves. This procedure has evolved over time to address valve migration, left ventricular outflow tract obstruction, and paravalvular leak. The authors present a refined technique that has been associated with the most reproducible outcomes. METHODS: A step-by-step description of the TMVR technique and outcomes of 8 patients treated using this technique are described. Baseline patient clinical and echocardiographic characteristics and 30-day post-TMVR outcomes are presented. RESULTS: Eight patients underwent transatrial TMVR at a single institution. Five had previous cardiac surgery. Mean STS score was 8%. Technical success by MVARC (Mitral Valve Academic Research Consortium) criteria was 100%. There was zero in-hospital and 30-day mortality. Procedural success by MVARC criteria at 30 days was 100%. Paravalvular leak immediately post-implant was none or trace in 6 and mild in 1. CONCLUSIONS: The technique described is reproducible and was associated with favorable outcomes in this early experience. It represents a useful technique for the treatment of mitral valve disease in the setting of severe annular calcification. A structured and defined implantation technique is critical to investigators as this field evolves.

Open Atrial Transcatheter Mitral Valve Replacement in Patients With Mitral Annular Calcification.

BACKGROUND: Mitral valve replacement in the setting of severe mitral annular calcification remains a surgical challenge. Transcatheter mitral valve replacement (TMVR) using an aortic balloon-expandable transcatheter heart valve is emerging as a potential treatment option for high surgical risk patients. Transseptal, transapical, or transatrial access is not always feasible, so an understanding of alternative implantation techniques is important. OBJECTIVES: The authors sought to present a step-by-step description of a contemporary transatrial TMVR technique using balloon-expandable aortic transcatheter heart valves. This procedure has evolved over time to address valve migration, left ventricular outflow tract obstruction, and paravalvular leak. The authors present a refined technique that has been associated with the most reproducible outcomes. METHODS: A step-by-step description of the TMVR technique and outcomes of 8 patients treated using this technique are described. Baseline patient clinical and echocardiographic characteristics and 30-day post-TMVR outcomes are presented. RESULTS: Eight patients underwent transatrial TMVR at a single institution. Five had previous cardiac surgery. Mean STS score was 8%. Technical success by MVARC (Mitral Valve Academic Research Consortium) criteria was 100%. There was zero in-hospital and 30-day mortality. Procedural success by MVARC criteria at 30 days was 100%. Paravalvular leak immediately post-implant was none or trace in 6 and mild in 1. CONCLUSIONS: The technique described is reproducible and was associated with favorable outcomes in this early experience. It represents a useful technique for the treatment of mitral valve disease in the setting of severe annular calcification. A structured and defined implantation technique is critical to investigators as this field evolves.

Aorta-innominate bypass through ministernotomy.

Atherosclerotic innominate artery occlusive disease can lead to cerebral and upper extremity ischemia. Innominate artery angioplasty and stenting can be complicated by stent fractures and restenosis; furthermore, this technique is limited in treatment of innominate artery occlusions. Ministernotomy to the second or third intercostal space can be used instead of conventional full sternotomy for open surgical revascularization of the innominate artery with excellent perioperative and long-term outcomes. This series of three consecutive patients highlights the technique of aorta-innominate artery bypass through ministernotomy.

Paravalvular regurgitation after conventional aortic and mitral valve replacement: A benchmark for alternative approaches.

OBJECTIVE: Paravalvular regurgitation is a known complication after transcatheter and sutureless aortic valve replacement. Paravalvular regurgitation also may develop in patients undergoing percutaneous mitral valve replacement. There are few studies on contemporary surgical valve replacement for comparison. We sought to determine the contemporary occurrence of paravalvular regurgitation after conventional surgical valve replacement. METHODS: We performed a single-center retrospective database review involving 1774 patients who underwent valve replacement surgery from April 2004 to December 2012: aortic in 1244, mitral in 386, and combined aortic and mitral in 144. Follow-up echocardiography was performed in 73% of patients. Patients with endocarditis were analyzed separately from noninfectious paravalvular leaks. Statistical comparisons were performed to determine differences in paravalvular regurgitation incidence and survival. RESULTS: During follow-up, 1+ or greater (mild or more) paravalvular regurgitation occurred in 2.2% of aortic cases and 2.9% of mitral cases. There was 2+ or greater (moderate or more) paravalvular regurgitation in 0.9% of aortic and 2.2% of mitral cases (P = .10). After excluding endocarditis, late noninfectious regurgitation 2+ or greater was detected in 0.5% of aortic and 0.4% of mitral cases (P = .93); there were no reoperations or percutaneous closures for noninfectious paravalvular regurgitation. CONCLUSIONS: In an academic medical center, the overall rate of paravalvular regurgitation is low, and late clinically significant noninfectious paravalvular regurgitation is rare. The benchmark for paravalvular regurgitation after conventional valve replacement is high and should be considered when evaluating patients for transcatheter or sutureless valve replacement.

Modified TandemHeart ventricular assist device for infant and pediatric circulatory support.

PURPOSE: The development of pediatric ventricular assist device (VAD) circuits with lower flow ranges for infants and small children is ongoing. We present our results with modifying a readily available adult VAD to support the pediatric population. DESCRIPTION: The TandemHeart VAD (CardiacAssist, Pittsburgh, PA) circuit was modified to include a variable restrictive recirculation shunt to permit lower flow ranges in small pediatric patients. EVALUATION: Initial benchtop flow rates and pressures were studied. Hemolysis trials were performed using whole bovine blood to compare plasma-free hemoglobin levels between modified and unmodified VAD circuits. The modified VAD was surgically implanted in 7 piglets (6 to 14 kg) and which supported them for 4 hours. Levels of hemolysis did not increase and full hemodynamic support was achieved. The modified TandemHeart VAD with a recirculation shunt was subsequently implanted in 2 pediatric patients who were bridged to transplant successfully. CONCLUSIONS: Because of its simplicity, availability, low prime volume, greater patient flow range, and lower cost, the modified TandemHeart VAD with a recirculation shunt should be considered as an alternative to extracorporeal membrane oxygenation and other pulsatile VADs in children.

Doubly committed and juxtaarterial ventricular septal defect: outcomes of the aortic and pulmonary valves.

BACKGROUND: The morphology of ventricular septal defects (VSDs) that are doubly committed and juxtaarterial places the patient at risk for aortic valvar prolapse and aortic valvar insufficiency (AI). Surgical repair of this type of defect often involves placing sutures through the base of one or more of the leaflets of the pulmonary valve, raising concern for late pulmonary valvar insufficiency (PI). The purpose of this review was to analyze the postoperative follow-up relating to potential late complications with the aortic and pulmonary valves. METHODS: Between 1980 and 2012, 106 patients with doubly committed juxtaarterial VSD underwent intracardiac repair. Median age at repair was 1.1 years. Preoperative evaluation showed 69 patients (65%) had aortic valvar prolapse and 51 (48%) had AI. Operative approach was through the pulmonary trunk in 88 (83%) of the patients. In 81 patients (76%), sutures securing the VSD patch had been placed through the base of the pulmonary valvar leaflets. RESULTS: Operative survival was 100%. Follow-up ranges from 6 months to 17 years, with a mean of 4.9 years. No patient had heart block or residual shunting. Of the 70 patients with long-term contemporary echocardiographic follow-up, 66 (94%) had trivial or no AI and 4 (6%) had mild AI. Of these patients, 49 (70%) had trivial or no PI, and 21 (30%) had mild PI. In 1 patient having aortic valvoplasty at the time of VSD closure, the aortic valve was replaced 7 months later. No other patient had worrisome progression of their AI or PI. CONCLUSIONS: The incidence of aortic valvar prolapse and AI in the setting of doubly committed juxtaarterial VSD is quite high. The optimal surgical approach is through the pulmonary trunk. Sutures placed through the base of the pulmonary valvar leaflets do not predispose to clinically significant late pulmonary valvar insufficiency. Timely surgical closure of this type of defect prevents progression of AI.

Reoperation after vascular ring repair.

The majority of patients having surgical intervention for a vascular ring have resolution of their symptoms. However, 5% to 10% of these patients develop recurrent symptoms related either to airway or esophageal compression and may require reoperation. In our series of 300 patients with vascular rings, we performed a reoperation on 26 patients, not all of whom were originally operated on at our institution. The four primary indications for reoperation were Kommerell diverticulum (n = 18), circumflex aorta (n = 2), residual scarring (n = 2), and tracheobronchomalacia requiring aortopexy (n = 4). All patients undergoing reoperation have had preoperative evaluation with bronchoscopy and computed tomographic scanning (CT) with 3-dimensional reconstruction. Patients with dysphagia have had a barium esophagram and esophagoscopy. Patients with a Kommerell diverticulum have undergone resection of the diverticulum and transfer of the left subclavian artery to the left carotid artery. The aortic uncrossing procedure has been used in patients with a circumflex aorta. Aortopexy has been used to treat anterior compression of the trachea by the aorta. Results of these reinterventions have been successful in nearly all cases. Lessons learned from these reoperations can be applied to prevent the need for reoperation by properly selecting the correct initial operation. A dedicated team caring for these children consisting of medical imaging, otolaryngology, cardiovascular-thoracic surgery, and critical care is imperative.

Right pulmonary artery to left atrial fistula in a neonate: case report and review of the literature.

A communication between the right pulmonary artery (RPA) and the left atrium is a rare congenital anomaly that presents with cyanosis and heart failure. We describe the surgical repair of an RPA to left atrial fistula using cardiopulmonary bypass in a neonate. Advanced imaging (computed tomography scan) guided the surgical approach. Although previous reports have associated a patent ductus arteriosus with high neonatal mortality, in our case, the ductus arteriosus was actually important for maintaining enough total pulmonary blood flow.

Left ventricular thrombus formation after repair of anomalous left coronary artery from the pulmonary artery.

Although thrombus formation following myocardial infarction in adults is well known, intracardiac thrombosis in children is uncommon. We report the case of a large left ventricular thrombus in an infant with ischemic cardiomyopathy secondary to anomalous origin of the left coronary artery from the pulmonary artery. Given its mobility and protrusion across the aortic valve, the patient underwent urgent thrombus removal through a transaortic approach. There were no embolic or neurologic complications. This case highlights that thrombectomy may be performed safely and successfully in critically ill pediatric patients.

Heart transplantation for the failing Fontan.

BACKGROUND: Patients with failing Fontan circulation are at high risk for complications after heart transplantation (HTx) because of multiple prior operations, elevated panel reactive antibody, hepatic dysfunction, coagulopathy, protein-losing enteropathy (PLE), and poor nutrition. The purpose of this review was to evaluate the outcome of HTx for these patients, including those who are status post-Fontan conversion. METHODS: Of 206 heart transplants at Ann & Robert H. Lurie Children's Hospital of Chicago from 1990 to 2012, 22 patients had a failing Fontan. Median age at HTx was 12.2 years, median interval from initial Fontan to HTx was 7.1 years. Potential preoperative risk factors included PLE (n = 15), mechanical ventilation (n = 8), prior Fontan conversion (n = 7), renal failure (n = 3), and plastic bronchitis (n = 2) Median number of prior operations was 3. Donor branch pulmonary arteries were used in 17 patients. RESULTS: There were 5 early deaths (23%), due to graft failure (1), pulmonary hypertension (1), and infection (3). There were 3 late deaths (13%) at 1, 5, and 8 years. Two of 3 patients with preoperative renal failure died. Survivors who had preoperative PLE (n = 11) and preoperative plastic bronchitis (n = 2) experienced complete resolution of these pathological conditions after heart transplantation. Median length of stay was 30 days. Five of 7 Fontan conversion patients survived, and 6 of 8 preoperative ventilator-dependent patients survived. One-, 5-, and 10-year survival was 77%, 66%, and 45%, respectively. CONCLUSIONS: The operative mortality of HTx for patients with a failing Fontan is high. Using the donor branch pulmonary arteries greatly facilitated the transplant. Because infection caused the majority of early deaths, lower intensity initial immunosuppression may be warranted. Transplantation was successful in treating PLE in all survivors. Prior Fontan conversion was not a risk factor. Preoperative mechanical ventilation was not a risk factor. Preoperative renal failure may be a relative contraindication. Earlier referral of failing Fontan patients may improve results.

Outcomes of surgical therapy for infective endocarditis in a pediatric population: a 21-year review.

BACKGROUND: Infective endocarditis is a rare disease in the pediatric population. We sought to define patient characteristics and outcomes of surgical therapy for endocarditis in children. METHODS: We performed a retrospective review of all patients with infective endocarditis who received surgical therapy between January 1, 1990, and March 1. 2011. We were interested in their congenital heart defect, prior surgical procedures, and outcome of the operation. RESULTS: We identified 35 cases of endocarditis in 34 patients requiring surgical intervention. Mean age was 10.7 ± 8.8 years. There was a bimodal age distribution at presentation: 11 (31%) were younger than 1 year and 15 (43%) were 10 to 21 years. Of the 34 patients, 22 (63%) had no history of prior cardiac operation. The infective organism was identified in 30 (86%), with Staphylococcus aureus (n = 8) and Streptococcus viridans (n = 6) predominating. Valve replacement was performed in 22 patients and valve repair in 10. All patients received 6 weeks of postoperative intravenous antimicrobial therapy. Operative mortality was 15% (5 of 34). The 5 deaths occurred in infants who were a mean age of 2.5 months, and 3 of the 5 infants (60%) were premature. Of 4 patients with fungal infection, 3 patients died. The Ross operation was performed successfully in 5 patients with severe aortic valve disease. Reoperations (n = 10 [28%]) included valve replacement in 5 and conduit replacement in 3, all but 1 due to somatic growth resulting in functional stenosis. CONCLUSIONS: The outcome of surgical therapy for endocarditis in children was similar to that reported for adults, with an overall mortality of 15%. The Ross operation was very effective in patients with aortic valve endocarditis. There is a significant incidence of late reoperation for valve and conduit replacement due to somatic growth. Age younger than 1 year, prematurity, and fungal organisms appear to be risk factors for death. Patients surviving to discharge had good outcomes, with no episodes of recurrent endocarditis.

Primary resection of Kommerell diverticulum and left subclavian artery transfer.

BACKGROUND: A Kommerell diverticulum (KD) is an aneurysmal remnant of the dorsal fourth aortic arch. This can be an independent cause of tracheoesophageal compression. We previously reported resection of the KD with left subclavian artery transfer to the left carotid artery for recurrent symptoms in patients with a right aortic arch, left ligamentum, and retroesophageal left subclavian artery after prior ligamentum division. In 2001 we began resecting the KD and transferring the left subclavian artery to the left carotid artery in selected patients as a primary operation. METHODS: From 2001 to 2011, 20 patients have had primary excision of a Kommerell diverticulum. Diagnosis was with computed tomographic scan (n = 14) or magnetic resonance imaging (n = 6) and bronchoscopy. Sixteen patients had a right aortic arch and 4 had a double aortic arch (right arch dominant). All patients were approached through a left thoracotomy. Fifteen patients had simultaneous division and reimplantation of the left subclavian artery into the left carotid artery. RESULTS: Mean age at operation was 9.1 ± 6.5 years (range 1.5 to 29.1 years). Symptoms included cough, wheezing, stridor, dysphagia, and dyspnea on exertion. Selection criteria included KD greater than 1.5 times the size of the left subclavian artery and posterior pulsatile compression of the trachea on bronchoscopy. There were no complications related to subclavian artery transfer. No patient required a blood transfusion. No patient had a recurrent laryngeal nerve injury or chylothorax. The mean hospital stay was 4.3 ± 2.5 days. All patients had resolution of their preoperative airway and esophageal symptoms. CONCLUSIONS: In selected patients with a vascular ring we now recommend resection of the associated Kommerell diverticulum and transfer of the retroesophageal left subclavian artery to the left carotid artery as a primary procedure. This strategy requires comprehensive and precise preoperative imaging with either computed tomography or magnetic resonance imaging.

Optimal initial palliation for patients with functionally univentricular hearts.

This review will outline the optimal, initial palliation for children who are born with a functionally univentricular heart. Optimizing the initial palliation is of critical importance in this patient population to prevent potential problems such as systemic outflow and pulmonary vein obstruction that may complicate further surgical intervention. The palliative techniques that are discussed include pulmonary artery banding, modified Blalock-Taussig shunt, Damus-Kaye-Stansel procedure, modified Norwood, hybrid, and early bidirectional Glenn. Our recommendations for optimal palliation for children with a univentricular heart are based on our experience with nearly 200 patients who had either a lateral tunnel or extracardiac Fontan procedure and 130 patients who had Fontan conversion with arrhythmia surgery.

Successful Bridge to Transplant Using the TandemHeart(R) Left Ventricular Assist Device in a Pediatric Patient.

A nine-year-old girl ( 23 kg) was successfully bridged to heart transplantation with the TandemHeart(®) centrifugal pump for 10 days. Although this cardiac assist device has been used in adults for short-term mechanical support, its use in the pediatric population has not been widely reported. The TandemHeart(®) was easy to implant, achieved appropriate flows in this pediatric patient, and allowed for extubation and ambulation while awaiting a donor heart.