Preserving the pulmonary valve in Tetralogy of Fallot repair: Reconsidering the indication for valve-sparing.

BACKGROUND: Tetralogy of Fallot (TOF) repair is a frequent procedure, and although valve-sparing (VS) repair is preferred, determining which patients can successfully undergo this operation remains controversial. We sought to identify parameters to determine a selective, accurate indication for VS repair. METHODS: We reviewed 71 patients (82%) undergoing VS repair. We analyzed hemodynamic data, intraoperative reports, and follow-up echocardiography results to identify acceptable indications. Patients requiring pulmonary valve (PV) reintervention versus no reintervention were compared. RESULTS: PV annulus size at repair was z-score of -2.0 (-5.3, 1.3). Approximately half (51%) had a z-score less than -2. Cox regression results showed this was not a risk factor for reintervention (p = .59). Overall, 1-, 3-, 5-, and 10-year freedom from PV reintervention rates were 95.8%, 92.8%, 91% and 77.8%, respectively. Residual pulmonary stenosis (PS) at initial repair was relatively higher in the reintervention group compared with no reintervention group (40 [28, 51] mmHg vs. 30 [22, 37] mmHg; p = .08). For patients with residual PS, pressure gradient (PG) was consistent over time across both groups (PV reintervention: -3 [-15, 8] mmHg vs. no reintervention: 0 [-9, 8] mmHg). The risk of PV reintervention is 3.7-fold higher when the PG from intraoperative TEE is greater than 45 mmHg (p = .04). CONCLUSIONS: Our review of the midterm outcomes of expanded indication for VS suggests intraoperative decision to convert to transannular patch is warranted if intraoperative postprocedure TEE PG is greater than 45 mmHg or RV pressure is higher than half of systemic pressure to prevent reintervention.

Pediatric aortic valve repair: Any development in the material for cusp extension valvuloplasty?

BACKGROUND: Aortic cusp extension is a technique for aortic valve (AV) repairs in pediatric patients. The choice of the material used in this procedure may influence the time before reoperation is required. We aimed to assess postoperative and long-term outcomes of patients receiving either pericardial or synthetic repairs. METHODS: We conducted a single-center, retrospective study of pediatric patients undergoing aortic cusp extension valvuloplasty (N = 38) with either autologous pericardium (n = 30) or CorMatrix (n = 8) between April 2009 and July 2016. Short- and long-term postoperative outcomes were compared between the two groups. Freedom from reoperation was compared using Kaplan-Meier analysis. Degree of aortic stenosis (AS) and aortic regurgitation (AR) were recorded at baseline, postoperatively, and at outpatient follow-up. RESULTS: At 5 years after repair, freedom from reoperation was significantly lower in the CorMatrix group (12.5%) compared to the pericardium group (62.5%) (p = .01). For the entire cohort, there was a statistically significant decrease in the peak trans-valvar gradient between preoperative and postoperative assessments with no significant change at outpatient follow-up. In the pericardium group, 28 (93%) had moderate to severe AR at baseline which improved to 11 (37%) postoperatively and increased to 21 (70%) at time of follow-up. In the biomaterial group, eight (100%) had moderate to severe AR which improved to three (38%) postoperatively and increased to seven (88%) at time of follow-up. CONCLUSION: In terms of durability, the traditional autologous pericardium may outperform the new CorMatrix for AV repairs using the cusp extension method.

Aortic root aneurysm repair in a neonate with Loeys-Dietz syndrome.

Loeys-Dietz syndrome is a connective tissue disorder known to cause aggressive aortopathy in paediatric patients, but it is extremely rare for cardiovascular events to present during infancy. We report the first successful aortic repair in a neonate with LDS presenting in extremis with an early onset, massive aortic aneurysm.

Pulmonary hypertension and mitral regurgitation in an infant with an anatomically normal mitral valve.

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.

Effects of Modified Ultrafiltration on Thromboelastographic Profile after Pediatric Cardiac Surgery.

Modified ultrafiltration (MUF) is still used after pediatric cardiopulmonary bypass (CPB) in some pediatric cardiac surgery centers to decrease transfusion requirements. Other potential benefits of MUF include clearance of inflammatory markers and improvement in myocardial function. Our hypothesis is that MUF will hemoconcentrate coagulation factors and improve thromboelastography (TEG) parameters after pediatric CPB. Patients younger than 6 months were prospectively enrolled over a year. TEG was carried out before MUF, after MUF, and after protamine administration. Paired t tests were conducted to compare values pre-MUF and post-MUF as well as post-MUF and post-protamine administration. Thirty patients were enrolled in the study, with 20 (67%) neonates in the cohort. Seven arterial switch operations and nine Norwood procedures were found to be performed among the cohort. Reaction time (R), angle (α), and maximum amplitude (MA) were significantly worse post-MUF compared with pre-MUF (p < .001). They improved significantly after protamine administration compared with post-MUF (p < .001). The amount of fluid removal was significantly associated with a worse post-MUF R, angle, and MA and worse post-protamine administration, angle, and MA but with no effect on post-protamine R. MUF caused worsening of TEG parameters that is reversed by protamine administration.

Post-cardiotomy Rescue Extracorporeal Cardiopulmonary Resuscitation in Neonates with Single Ventricle After Intractable Cardiac Arrest: Attrition After Hospital Discharge and Predictors of Outcome.

Extracorporeal cardiopulmonary resuscitation (ECPR) in children with cardiac arrest refractory to conventional cardiopulmonary resuscitation (CPR) has been reported with encouraging results. We reviewed outcomes of neonates with functional single ventricle (FSV) surviving post-cardiotomy ECPR after hospital discharge. Fifty-eight patients who required post-cardiotomy extracorporeal membrane oxygenation (ECMO) since the introduction of our ECPR protocol (January 2007-December 2011) were identified. Forty-one were neonates. Survival analysis was conducted. Of 41 neonates receiving post-cardiotomy ECMO, 32 had FSV. Twenty-one had ECPR. Fourteen underwent Norwood operation (NO) for hypoplastic left heart syndrome (HLHS). Seven had non-HLHS FSV. Four (of 7) underwent modified NO/DKS with systemic-to-pulmonary shunt (SPS), 2 SPS only and 1 SPS with anomalous pulmonary venous connection repair. Mean age was 6.8 ± 2.1 days. ECMO median duration was 7 days [interquartile range (IQR25-75: 4-18)]. Survival to ECMO discontinuation was 72% (15 of 21 patients) and at hospital discharge 62% (13 of 21 patients). The most common cause of late attrition was cardiac. At last follow-up (median: 22 months; IQR25-75: 3-36), 47% of patients were alive. Duration of ECMO and failure of lactate clearance within 24 h from ECMO deployment determined late survival after hospital discharge (p < 0.05). Rescue post-cardiotomy ECMO support in neonates with FSV carries significant late attrition. ECMO duration and failure in lactate clearance after deployment are associated with unfavorable outcome. Emphasis on CPR quality, refinement of management directives early during ECMO and aggressive early identification of patients requiring heart transplantation might improve late survival.

Evolution of the surgical approach to congenitally corrected transposition of the great arteries.

The traditional surgical approach (physiologic repair) of congenitally corrected transposition of the great arteries (ccTGA) attempts at restoring normal physiology by repairing the associated lesions. It fails to address the most serious anatomic abnormality, mainly ventriculoarterial discordance, and results in less than optimal long-term outcomes. Anatomic repair was introduced to incorporate the left ventricle into the systemic circulation. The excellent short-term and intermediate results of the double switch operation and its modifications make it the procedure of choice for the treatment of ccTGA.

Fate of ventricular and valve performance following early bidirectional Glenn procedure after Norwood operation controlled for hypoplastic left heart syndome anatomic subtype.

The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS-MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS-MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7-4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS-MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.

Tetralogy of Fallot with atrioventricular septal defect: surgical strategies for repair and midterm outcome of pulmonary valve-sparing approach.

Repair for tetralogy of Fallot (TOF) with complete atrioventricular septal defect (CAVSD) has been reported with good early and intermediate outcomes. Morbidity, however, remains significantly high. To date, repair of CAVSD/TOF using a pulmonary valve-sparing technique (PVS) and freedom from valve reoperation are not well defined. A study was undertaken to investigate outcomes. This study was conducted in as a retrospective investigation. Between January 1988 and December 2008, 13 consecutive patients with CAVSD/TOF were identified, and their records were reviewed retrospectively. Of these 13 patients, 9 had Rastelli type C CAVSD. Trisomy 21 was present in 9 cases (69 %; 7 with type C). Five patients had received a systemic-to-pulmonary shunt (SPS) before complete repair at a mean age 1.7 ± 0.6 months. All the patients survived until complete repair. At complete CAVSD/TOF repair, AVSD was corrected with a two-patch technique in all patients. For eight patients (61.5 %), PVS was used. The remaining five patients had transannular patch (TAP) repair. The mean age at complete repair was 6.3 ± 2.4 months. At complete repair, the mean cardiopulmonary bypass time was 173.5 ± 30.6 min, and the cross-clamp time was 134.7 ± 28.8 min. There was one hospitalization and no late deaths. The median follow-up period was 9.2 years [interquartile range (IQR), 4.7-13.3 years]. The actuarial survival was 90.0 ± 9.5 % at 1 year, 90 ± 9.5 % at 5 years, and 90 ± 9.5 % at 8 years. Of the 12 survivors, 6 had some reintervention during the follow-up period. Within the first 11 years after complete repair, two patients underwent left atrioventricular (AV) valve repair, and one patient had right AV valve repair. Two patients had residual VSD closure. Four patients underwent the first right ventricular outflow tract (RVOT) reintervention for critical insufficiency or stenosis at a mean interval of 6 ± 21) months. One patient had a second RVOT reoperation. Findings showed that CAVSD/TOF with PVS was related to significantly higher freedom from RVOT reintervention (100 % at 1, 5, and 8 years compared with 80 ± 17.9 % at 1 year, 60 ± 21.9 % at 5 years, and 40 ± 21.9 % at 8 years for CAVSD/TOF using TAP; P < 0.05). No patient who underwent PVS had left ventricular outflow tract obstruction requiring reoperation. Overall freedom from any reintervention was 90.9 ± 8.6 % at 1 year, 71.6 ± 14.0 % at 5 years, and 53.7 ± 8.7 % at 8 years in this group of patients. Correction of TOF with CAVSD can be performed at low risk with favorable intermediate-term survival and satisfactory freedom from reoperation. Use of TAP can be avoided in almost two thirds of patients and may influence freedom from early RVOT reintervention.

Aortic Thrombosis and Subsequent Myocardial Infarction in a Previously Healthy 12-Year-Old Male.

Aortic thrombus formation in children is uncommon, particularly in an otherwise healthy pediatric patient. Thromboembolism of such thrombi resulting in subsequent ST-segment elevation myocardial infarction is, thus, exceedingly rare. (Level of Difficulty: Intermediate.).

Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation.

Aortic atresia-mitral stenosis (AA-MS) has been implicated as a determinant of outcome after Stage-1 palliation (S1P) in hypoplastic left heart syndrome (HLHS).Studies evaluating the association of AA-MS with ventriculo-coronary connections (VCC) and mortality report conflicting results. The significance of VCC, myocardial protection, and shunt strategy after S1P has yet to be determined. Between January 2005 and July 2009, 100 neonates with HLHS underwent S1P. Mitral and aortic valves and presence of VCC were assessed. Antegrade continuous cold blood cardioplegia was administered throughout the vast extent of the neo-aortic reconstruction. A right ventricle-to-pulmonary shunt was used for an ascending aortic diameter of 0.6 mm/kg or less. Survival analysis was performed to determine predictors and assess impact of AA-MS and VCC on hospital and interstage mortality. Twenty-seven (of 100) patients had AA-MS. The mean age and weight at S1P were 6.5 ± 2.8 days and 3.09 ± 0.47 kg, respectively. VCC were found in 56% of AA-MS. Twenty-two had Norwood-Sano, 3 had classic Norwood, and 2 had hybrid S1P. VCC were associated with AA-MS, endocardial fibroelastosis, and ascending aortic size <2 mm (P < 0.05) but not higher mortality (P = ns). Operative and interstage survival for AA-MS after S1P was 85.2 and 71%, respectively (not statistically different compared to all other subtypes; P = ns). Actuarial survival after S1P at 1, 3, 6, 12, and 36 months was 92.9 ± 4.9, 78.6 ± 7.8, 75 ± 8.2, 71.3 ± 8.3, and 71.3 ± 8.3%, respectively. Intact atrial septum and post-S1P renal dysfunction (P < 0.05) were independent predictors of hospital and interstage mortality. In patients with HLHS, AA-MS carries no survival disadvantage after S1P during the hospital and interstage period regardless of VCC. Intact atrial septum and post-S1P renal dysfunction predict early and interstage mortality. Myocardial protection and shunt strategy might influence the outcome in this HLHS variant.

Technical Modifications That Might Improve Long-term Outcomes of the Ross Procedure in Children.

BACKGROUND: Failure of the pulmonary valve autograft (PVA) after the Ross procedure (RP) has discouraged its widespread use and has led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. METHODS: Sixty-nine patients (median age, 12 years; range, 0.25-17.9) underwent the RP between January 1996 and December 2018. A concomitant Konno procedure was performed on 20 of 69 patients (29%). Prior interventions included balloon valvuloplasty in 30 (44%) and/or surgical valvuloplasty in 39 (57%). Technical modifications included using the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction, and wrapping the native root remnant around the PVA. RESULTS: Operative mortality was 1 of 69 patients (1.5%), with no late deaths. No patient had neoaortic valvar stenosis and 7 of 68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median, 9.4 years; range, 0.4-21.3) there was no significant change in Z scores of annulus, sinus, or sinotubular junction diameters when compared with those at discharge. Three patients (4.4%) required late autograft replacement, 2 PVA repair, and 2 resection of a pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 years. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80%, respectively. CONCLUSIONS: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients.

Congenitally Abnormal Aortic Valve Causing Coronary Obstruction and Cardiac Arrest in Infancy.

Many instances of coronary artery anomalies are documented in the literature; however, the detection and treatment of an asymmetric, large aortic cusp causing obstruction of a coronary ostium in a symptomatic infant remains unreported. We present a case of a 2-month-old infant with an enlarged right coronary cusp obstructing the left coronary ostium, requiring emergent repair by relocating the left coronary button and reconstructing the sinus of Valsalva with autologous pericardium. This procedure preserved native aortic valve function, and the child remains asymptomatic months after discharge.

Outcomes From Three Decades of Infant and Pediatric Heart Transplantation.

Infants are a unique transplant population due to a suspected immunologic advantage, in addition to differences in size and physiology. Consequently, we expect infants to have significantly different diagnoses, comorbidities, and outcomes than pediatric transplant recipients. In this study, we compare patterns and trends in pediatric and infant heart transplantation during three decades. The United Network for Organ Sharing (UNOS) database was queried for transplants occurring between January 1990 and December 2018. Patients were categorized as pediatric (1-17) or infant (0-1). Congenital heart disease (CHD) primary diagnoses have increased from 37% to 42% in pediatric patients (p = 0.001) and decreased from 80% to 61% in infants during the 1990s and 2010s (p < 0.001). Those with CHD had worse outcomes in both age groups (p < 0.001). Infants who underwent ABO-incompatible transplants had similar survival as compared to those with compatible transplants (p = 0.18). Overall, infants had better long-term survival and long-term graft survival than pediatric patients; however, they had worse short-term survival (p < 0.001). Death due to rejection or graft failure was less likely in infants (p = 0.034). However, death from infection was over twice as common (p < 0.001). In summary, pediatric and infant heart transplant recipients differ in diagnoses, comorbidities, and outcomes, necessitating different care for these populations.

Pericardial effusion in a preterm infant resulting from umbilical venous catheter placement.

Pericardial effusion in neonates is a rare occurrence associated with malpositioning of central venous catheters. This report describes a case of pericardial effusion in which echocardiographic determination of line position, typically considered one of the most reliable means of placement verification, was misleading. The infant ultimately did well after pericardiocentesis, with complete resolution of symptoms and no further complications.

Ventriculocoronary artery connections with the hypoplastic left heart: a 4-year prospective study: incidence, echocardiographic and clinical features.

Ventriculocoronary connections (VCCs), also called sinusoids, occur with hypoplastic left heart (HLH). Previous reports are limited to case reports, pathologic series, and surgical series with limited detail, which may underestimate the incidence and overestimate the severity of VCCs in HLH. A study was conducted to determine the incidence VCCs in HLH, their effect on survival, and their echocardiographic and clinical features. The echocardiograms and medical records of 100 consecutive neonatal HLH cases were analyzed. All had an aortic and a mitral valve diameter and a left ventricular (LV) volume less than Z-3. For palliation, Norwood, Sano, or hybrid procedures were used, and if the patient was alive, subsequent bidirectional Glenn and extracardiac Fontan procedures were applied. Cases were classified as manifesting mitral and aortic atresia (MAAA), mitral and aortic stenosis (MSAS), or mitral stenosis and aortic atresia (MSAA). All other diagnoses or any case with additional cardiac anomalies were excluded from the study. Overall, VCCs were found in 15% of the cases. They occurred in 56% of the MSAA subtype cases and were not statistically associated with a high mortality rate. However, in one case, large and multiple VCCs definitely caused or contributed to early death. All VCCs had a transmyocardial course, a turbulent color-Doppler flow, and a dominant usually retrograde systolic coronary artery flow pattern. The VCCs were associated (p < 0.05) with MSAA, endocardial fibroelastosis, and ascending aortic size less than 2 mm. As shown by the findings, 15% of the HLH patients had MSAA with VCCs. Unless the VCCs were large or extensive, they did not contribute to mortality. Detailed echocardiographic analysis of VCCs in HLH was feasible. Recent reports emphasize more severe cases.

A Unique Case of Middle Aorta Syndrome With a "Corkscrew" Descending Aorta.

Middle aortic syndrome is a rare anatomic defect of the descending aorta and presents with hypertension. We present a unique case with "corkscrew" configuration. This case highlights the need for evaluation with advanced imaging for diagnosis to facilitate surgical management and avoid hypertensive complications.

Acquired Cor Triatriatum Sinister After the Fontan Operation and Successful Resection in a Child.

Cor triatriatum sinister is a rare congenital lesion encountered in children. It consists of a fibromuscular membrane that separates the left atrium into two chambers resulting in a triatrial heart and often occurs with other structural cardiac anomalies. The acquired form is uncommon and has only been reported after orthotopic heart transplantation or as a complication of infective endocarditis in adults. These cases were mostly because of hypertrophied atrial tissue or suture lines and torsion of the atrium. We describe the first case of acquired cor triatriatum late after the Fontan procedure with successful surgical resection in a child.

Improved Results of Aortic Arch Reconstruction in the Norwood Procedure.

BACKGROUND: Despite several surgical modifications, the reported incidence of neoaortic coarctation after stage 1 palliation (S1P) of hypoplastic left heart syndrome remains high. A modification of aortic arch reconstruction that may decrease the incidence of neoaortic coarctation after S1P is described. METHODS: Between January 2008 and May 2013, 114 patients with hypoplastic left heart syndrome underwent S1P. The hospital records of the 101 surviving patients (89%) were reviewed. Demographic data and perioperative variables for S1P were collected. A modified technique for aortic arch reconstruction that contains a bovine pericardial patch tailored and sutured in a specific way was used in 51 patients (group MT), whereas the traditional technique was used in 50 patients (group TT). Clinical echocardiographic and cardiac catheterization data were analyzed to determine the incidence and location of neoaortic coarctation. RESULTS: There were no significant differences in the baseline characteristics, the intraoperative course, or hospital mortality between the two groups of subjects. Duration of ventilation, intensive care unit stay, and hospitalization were significantly shorter in group MT. The mean follow-up was 2.02 ± 0.63 years and 3.98 ± 0.66 years in each group, respectively. Coarctation developed in 4 of 51 patients (7.8%) in group MT compared with 10/50 patients (20%) in group TT. Multivariable logistic regression analysis showed that the estimated odds of coarctation for group MT were 0.41 (95% confidence interval: 0.04, 4.32) times the estimated odds of coarctation for group TT. CONCLUSIONS: The described technique may decrease the incidence of recoarctation after S1P by minimizing aortic arch and descending aorta distortion and providing ample enlargement of the aorta at its narrowest diameter.