Post-operative anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery.

Morgagni hernias account for less than 5% of congenital diaphragmatic hernias. They are characteristically retrosternal and bilateral, with right-sided predominance. An association between Trisomy 21 and diaphragmatic hernias resembling Morgagni hernia has been reported, but the effect of cardiac surgery on its formation has not been investigated. The purpose of this study was to determine whether there is a higher incidence of anterior diaphragmatic hernias in children with Trisomy 21 after cardiac surgery. We compared the prevalence of anterior diaphragmatic hernias in 92 patients with Trisomy 21 who underwent cardiac surgery with its prevalence in 100 children without Trisomy 21 who underwent cardiac surgery. All available CXRs of all children underwent revision for the presence of an anterior diaphragmatic hernia by a pediatric radiologist. Within the study group, four cases of an anterior diaphragmatic hernia were detected, all upon presentation to the emergency room due to breathing difficulties. No cases of an anterior diaphragmatic hernia were found in the control group (P = 0.0094). CONCLUSIONS: A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing difficulty. If CXR findings are uncertain, UGI series and\or CT should be performed. In light of our findings, the surgical technique has been modified in patients with DS in our medical center. WHAT IS KNOWN: • Several studies reported an association between Trisomy 21 and diaphragmatic hernia resembling Morgagni hernia, but the effect of cardiac surgery on its formation has not been investigated. WHAT IS NEW: • There is a higher incidence of anterior diaphragmatic hernia resembling a Morgagni hernia in children with Trisomy 21 after cardiac surgery. • A high index of suspicion for an anterior diaphragmatic hernia should be maintained in children with Trisomy 21 who have undergone cardiac surgery and present with breathing difficulty. If CXR findings are uncertain, UGI series and\or CT should be performed.

A DNA methylation atlas of normal human cell types.

DNA methylation is a fundamental epigenetic mark that governs gene expression and chromatin organization, thus providing a window into cellular identity and developmental processes1. Current datasets typically include only a fraction of methylation sites and are often based either on cell lines that underwent massive changes in culture or on tissues containing unspecified mixtures of cells2-5. Here we describe a human methylome atlas, based on deep whole-genome bisulfite sequencing, allowing fragment-level analysis across thousands of unique markers for 39 cell types sorted from 205 healthy tissue samples. Replicates of the same cell type are more than 99.5% identical, demonstrating the robustness of cell identity programmes to environmental perturbation. Unsupervised clustering of the atlas recapitulates key elements of tissue ontogeny and identifies methylation patterns retained since embryonic development. Loci uniquely unmethylated in an individual cell type often reside in transcriptional enhancers and contain DNA binding sites for tissue-specific transcriptional regulators. Uniquely hypermethylated loci are rare and are enriched for CpG islands, Polycomb targets and CTCF binding sites, suggesting a new role in shaping cell-type-specific chromatin looping. The atlas provides an essential resource for study of gene regulation and disease-associated genetic variants, and a wealth of potential tissue-specific biomarkers for use in liquid biopsies.

The Relationship Between Body Mass Index and In-Hospital Mortality in Patients Following Coronary Artery Bypass Grafting Surgery.

Background: The association between Body Mass Index (BMI) and clinical outcomes following coronary artery bypass grafting (CABG) remains controversial. Our objective was to investigate the real-world relationship between BMI and in-hospital clinical course and mortality, in patients who underwent CABG. Methods: A sampled cohort of patients who underwent CABG between October 2015 and December 2016 was identified in the National Inpatient Sample (NIS) database. Outcomes of interest included in-hospital mortality, peri-procedural complications and length of stay. Patients were divided into 6 BMI (kg/m2) subgroups; (1) under-weight ≤19, (2) normal-weight 20-25, (3) over-weight 26-30, (4) obese I 31-35, (5) obese II 36-39, and (6) extremely obese ≥40. Multivariable logistic regression model was used to identify predictors of in-hospital mortality. Linear regression model was used to identify predictors of length of stay (LOS). Results: An estimated total of 48,710 hospitalizations for CABG across the U.S. were analyzed. The crude data showed a U-shaped relationship between BMI and study population outcomes with higher mortality and longer LOS in patients with BMI ≤ 19 kg/m2 and in patients with BMI ≥40 kg/m2 compared to patients with BMI 20-39 kg/m2. In the multivariable regression model, BMI subgroups of ≤19 kg/m2 and ≥40 kg/m2 were found to be independent predictors of mortality. Conclusions: A complex, U-shaped relationship between BMI and mortality was documented, confirming the "obesity paradox" in the real-world setting, in patients hospitalized for CABG.

Outcomes of sublobar resection vs lobectomy for invasive clinical stage T1N0 non-small-cell lung cancer: A propensity-match analysis.

BACKGROUND: The role of sub lobar resection (SLR; either segmentectomy or wedge resection) vs lobectomy (LBCT) for invasive clinical stage T1N0 non-small-cell-lung-cancer (NSCLC) has not been fully established yet. AIM: We aimed to characterize the preoperative parameters leading to selecting SLR and compare the overall survival (OS) and disease-free survival (DFS) of these two surgical approaches. METHODS: Clinical data on 162 patients (LBCT-107; SLR-55) were prospectively entered in our departmental database. Preoperative parameters associated with the performance of SLR were identified using univariate and multivariate cox regression analysis. The Kaplan-Meier method was used to compute OS and DFS. Comparison between LBCT and SLR groups and 32 propensity-matched groups was performed using Log-rank test. RESULTS: Median follow-up time for the LBCT and SLR groups was 4.76 (Inter-quartile range [IQR] 2.96 to 8.23) and 3.38 (IQR 2.9 to 6.19) years respectively. OS and DFS rates were similar between the two groups in the entire cohort (OS-LBCT vs SLR P = .853, DSF-LBCT vs SLR P = .653) and after propensity matching (OS-LBCT vs SLR P = .563 DSF-LBCT vs SLR P = .632). Specifically, Two- and five-year OS rates for LBCT and SLR were 90.6.% vs 92.7%, 71.8% vs 75.9% respectively. Independent predictors of selecting for SLR included older age (P < .001), reduced FEV1% (P = .026), smaller tumor size (P = .025), smaller invasive component (P = .021) and higher American Society of Anesthesiology scores (P = .014). CONCLUSIONS: In 162 consecutive and 32 matched cases, SLR and lobar resection had similar overall and disease-free survival rates. SLR may be considered as a reasonable oncological procedure in carefully selected T1N0 NSCLC patients that present with multiple comorbidities and relatively small tumors.

Long-Term Aortic Valve Function in Patients With or Without Surgical Treatment for Discrete Subaortic Stenosis.

The purpose of this study was to evaluate the long-term function of the aortic valve in patients with discrete subaortic stenosis (DSS), and its prognostic implications. 34 patients with the diagnosis of isolated DSS were treated at our pediatric heart center between 1992 and 2019. Demographic, echocardiographic and surgical data were analyzed. 26 patients had surgery to remove the subaortic membrane. The primary endpoint of this study was a change in the aortic valve function, secondary outcomes included functional class, aortic valve replacement, and reoperation due to recurrence of DSS. The mean post-operative follow-up time was 7.07 years (1-22.5). At the time of the most recent follow up, in the surgical group 11 patients (46%) had echocardiographic evidence of deterioration in their aortic valve insufficiency (AI) (p < 0.002). The deterioration was associated with a pre-operative left ventricular outflow tract (LVOT) gradient greater than 49 mmHg (p < 0.022). 8 patients with DSS were followed for a mean of 12.9 years (6.8-21.3), without meeting the criteria for surgical intervention, none showed any change in the aortic valve function during follow-up time. DSS resection may not prevent worsening of aortic valve insufficiency over time. Patients with stable AI and low LVOT gradient may not require surgery. Given these findings, it may be appropriate to carefully follow patients with new onset AI and low LVOT gradient, delaying surgical intervention.

Senning With Aortic Translocation and Anatomic Repair for Congenitally Corrected Transposition.

BACKGROUND: Anatomic repair for congenitally corrected transposition of the great arteries with ventricular septal defect (VSD) and pulmonic stenosis has been accomplished with atrial switch and Rastelli. Aortic translocation offers a direct left ventricular outflow without an extraanatomic right ventricular-to-pulmonary conduit, which may lead to decreased reoperations. We reviewed our entire experience performing Senning with aortic translocation (SAT). METHODS: From 2007 to 2017, 8 patients (mean age, 14.1 months; size, 8.86 kg) underwent SAT. Associated anomalies included situs inversus (n = 2), dextrocardia (n = 6), multiple muscular VSDs (n = 2), abnormal or straddling atrioventricular valve chords (n = 5), and branch pulmonary artery stenosis (n = 3). Four of 8 had previous systemic arterial shunts. Mean cardiopulmonary bypass was 487 minutes, and mean cardiac ischemic time was 307 minutes. Additional procedures included repair of branch pulmonary artery stenoses and closure of multiple muscular VSDs. RESULTS: There was no hospital death. One patient was supported with extracorporeal membrane oxygenation because of junctional tachycardia on postoperative day 5. One patient required pacemaker placement for first-degree heart block. Median hospital length of stay was 31 days. Mean length of follow-up was 52 months. All patients remain well with mild or no aortic regurgitation. The first patient underwent a repeat surgical operation for pulmonary venous baffle obstruction 2 years after SAT. CONCLUSIONS: Despite the technical complexity, patient outcomes have been satisfactory. We believe SAT provides a superior anatomic repair in these complex defects. Longer-term follow-up is needed regarding late intervention.

Surgical Treatment of Palestinian Patients With Congenital Heart Disease in a Medical Center in Israel: Challenges and Outcome.

BACKGROUND: The treatment of congenital heart disease patients in the West Bank and Gaza involves both medical and political challenges. Understanding the difficulties faced in treating the Palestinian population is an important step to improving surgical care, better allocating resources and overcoming the region's unique problems. METHODS: The Hadassah Medical Center congenital heart disease database over the 2011-2017 period was analyzed. There were 872 operations performed in patients with Israeli health insurance and 207 operations in Palestinian patients. Patient characteristics and surgical outcome were compared between the two groups using standard statistical practices. FINDINGS: The Society of Thoracic Surgeons Complexity Scores were significantly higher in the Palestinian patients, p = 0.003 (d = 0.27, 95% CI, 0.12 to 0.42). Israeli neonates had surgery at an average age of 9.5 ±â€¯7.8 days as compared to Palestinian neonates with an average age of 15.7 ±â€¯8.2 days, p < 0.001 (d = 0.78, 95% CI, 0.41 to 1.15), a finding indicative of a possible delay of treatment. Overall in hospital mortality was not significantly different. Late mortality was significantly higher for the Palestinian 5.4% (9/168) compared to Israeli patients 2% (14/698), p = 0.015 (RR = 2.67, 95% CI, 1.18 to 6.07). INTERPRETATION: The findings suggest that Palestinian patients receive later treatment and poorer follow-up care than Israeli patients. Despite the political challenges in the region surgical results are excellent and comparable between the two groups. The challenges described are not unique to congenital heart disease and may affect many medical fields. We believe that extensive collaborations between Israeli and Palestinian physicians may be key to improving the Palestinian medical care. FUNDING: None.

Midterm outcomes of right ventricular outflow tract reconstruction using the Freestyle xenograft.

OBJECTIVE: Various options exist for right ventricular outflow tract (RVOT) reconstruction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined. DESIGN: We performed a retrospective review of all non-Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by surgery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression. RESULTS: Between January 2002 and December 2015, there were 182 patients identified. Sixteen patients were lost to follow-up and 3 patients died, unrelated to cardiac surgery. Of the remaining 163 patients, the median age was 12.2 years (interquartile range 6.4-16.4), median weight was 39.0 kg (interquartile range 19.9-59.3), and the median body surface area was 1.23 m2 (interquartile range 0.79-1.64). Ninety-three (57%) patients had tetralogy of Fallot. The median follow-up was 5.4 years (interquartile range 2.9-8 years). There were no operative or cardiac-related deaths. Thirty-eight patients (23%) required reintervention. The rate of freedom from reintervention was 93.2% (95% CI 86.7%-96.6%) at 5 years and 48.4% (95% CI 34.9%-60.6%) at 10 years. Age < 10 years, weight < 39 kg, and body surface area <1.2 m2 at the time of valve placement, as well as valve size ≤25 mm were significantly associated with need for earlier reintervention. CONCLUSIONS: The Freestyle root in the RVOT is associated with excellent survival and low midterm need for reintervention. Its longevity is comparable to published data on homografts and other bioprosthetic valves.

Radical multidisciplinary approach to primary cardiac sarcomas.

OBJECTIVES: Primary cardiac sarcomas are extremely rare, but aggressive, tumours. The median survival with conventional treatment is 6-12 months. Recent data suggest that a radical multidisciplinary approach may improve patient outcome. We sought to evaluate our institutional experience with these tumours. METHODS: A multidisciplinary cardiac tumour programme was established 3 years ago based on the experience and support of our collaborating institution. Treatment consisted of pre- and postoperative chemotherapy, complete (R0) resection of the tumour with structural reconstruction and radiation therapy in selected cases. Left atrial tumours were resected using the cardiac autotransplantation technique. Bovine pericardium was used to reconstruct free-chamber walls or the septum. Valves were replaced by bioprostheses. A variety of autologous, allogeneic and synthetic vascular grafts were used to reconstruct the aorta, pulmonary arteries (PAs) and coronary arteries. RESULTS: Seven patients (3 males), age 51 ± 11 years (35-63), underwent eight operations. Tumour sites were PAs in 2 patients, left atrium in 3, right atrium in 2 and both great vessels in 1. Complete resection was achieved in all cases. There was no operative mortality. Two patients required implantation of a permanent pacemaker. Median survival was 24 months. Three patients died of metastatic disease and 1 sudden death 7, 23, 31 and 33 months after diagnosis. Three patients are alive at 2, 8 and 33 months, in functional Class I or II. One patient developed tumour recurrence and 2 have no evidence of disease. CONCLUSIONS: A radical multidisciplinary approach to cardiac sarcomas consisting of multimodality treatment and complex, technically demanding surgery, is safe and markedly improves (doubling) patient survival.

Valve-sparing aortic root replacement for patients with a Fontan circulation.

BACKGROUND AND AIM OF THE STUDY: Aortic root aneurysm is an emerging disease related to the treatment of patients with hypoplastic left heart syndrome (HLHS) and other single-ventricle physiology. The authors' experience with valve-sparing aortic root replacement and concomitant procedures in patients with a single ventricle was reviewed. METHODS: Four patients aged 11 to 19 years presented with progressive aortic root dilatation. All had undergone a Fontan completion: two patients for HLHS, one patient for tricuspid valve atresia, and one for pulmonary atresia with a ventricular septal defect. Two patients developed more than grade 2+ aortic valve insufficiency, and one patient developed symptomatic left anterior descending coronary artery compression. The preoperative root dimension ranged from 38 to 56 mm (mean 45mm). A valve-sparing aortic root replacement, annular reduction and ascending aorta replacement was performed in all patients. Two patients underwent a Fontan revision, two an aortic valve repair, and one patient a tricuspid valve repair. RESULTS: At a mean follow up of 2.6 years, all four patients were in NYHA functional class I. Two patients underwent neo-aortic valve replacement at about two years after aortic root replacement and valve repair, as they had developed severe neo-aortic valve insufficiency. Both of these patients had HLHS. In the other two patients, the most recent echocardiography demonstrated grade 0 to 1+ aortic insufficiency, with good function of the single ventricle. CONCLUSION: Aortic root reimplantation is an effective treatment for aortic root dilatation in patients with a single ventricle. However, if associated with significant aortic insufficiency, then a long-term freedom from valve replacement is questionable.

Outcome in infants less than 3 kilograms for placement of saphenous venous homografts as systemic-to-pulmonary arterial shunts.

BACKGROUND: Establishing stable and adequate flow of blood to the lungs using a systemic-to-pulmonary arterial shunt in infants with low birth weight may involve significant morbidity and mortality. We reviewed our experience with this procedure in patients weighing less than 3 kilograms. METHODS: Between June, 2002, and June, 2007, we placed systemic-to-pulmonary arterial shunts in 32 infants weighing less than 3 kilograms, the range being 1.8 to 2.86 kg, with a median of 2.5 kg. The median age at placement of the shunt was 8 days, with a range from 2 to 70 days. In 17 patients (53%), the anatomic defects had produced a functionally univentricular heart, while 15 (47%) had defects which permitted staging to biventricular repair. Patients staged to univentricular palliation were much more likely to have a circulation dependent on the arterial duct as compared with those staged to biventricular palliation (p < 0.001). The latter patients tended to have smaller pulmonary arteries, significantly the left pulmonary artery, which has a median diameter of 3.6 versus 2.0 mm, p = 0.01. In all patients a saphenous venous homograft was used as the conduit, its size ranging in diameter from 2.5 to 4 mm, with a median of 3.0 mm. RESULTS: The overall hospital mortality rate for the entire cohort was 6.25%, with 2 patients dying. There was no significant difference between the two groups with regard to length of stay in intensive care or hospital. Follow-up has ranged from 3 months to 4.7 years, with a mean of 2.1 years). Of those with functionally univentricular hearts, 3 have subsequently died, along with 1 patient having a biventricular circulation (p = 0.3). All deaths occurred before takedown of the shunt. A trend toward longer survival was noted in those with biventricular as compared to functionally univentricular circulations (p = 0.06). CONCLUSION: Systemic-to-pulmonary arterial shunts can be constructed safely in infants with biventricular physiology born with low weight. Those having functionally univentricular circulations carry an increased rate of mortality for the period of shunting. Using the saphenous venous homograft permits use of smaller grafts, which does not significantly increase the risk for thrombosis or survival when compared to previous studies using polytetrafluoroethylene grafts.

Anomalous coronary artery with aortic origin and course between the great arteries: improved diagnosis, anatomic findings, and surgical treatment.

BACKGROUND: Anomalous origin of a coronary artery with subsequent coursing between the great vessels is a rare congenital heart defect that may cause myocardial ischemia and sudden death. Because of the fatality rate of this anomaly, many are diagnosed at the postmortem examination, and reports of surgical correction are few. We present our experience with the diagnosis and surgical treatment of this rare coronary anomaly. METHODS: Between June 2003 and August 2005, 9 patients (8 males) were diagnosed with anomalous origin of a coronary artery coursing between the great vessels. The mean age was 12 +/- 5.8 years (range, 4 months to 23 years). Three patients had an intramural origin of the coronary artery. One infant had a single coronary artery and was diagnosed during follow-up of other heart defects. The 8 older patients all presented with exertional syncope or chest pain and echocardiography was used for the initial diagnosis. Six patients had coronary artery reimplantation in the correct aortic sinus, 2 patients had unroofing of the intramural coronary segment, and 1 patient had pericardial patch enlargement of his right coronary artery before reimplantation. One patient, the infant, awaits repair at an older age. RESULTS: No postoperative deaths occurred. The mean hospital stay was 5.5 +/- 1.2 days (range, 4 to 8 days). Three patients had transient ST segment changes during the first 24 hours postoperatively. Follow-up was 4 months to 2.5 years. All patients underwent an exercise myocardial perfusion scan 3 months postoperatively without evidence of myocardial ischemia, and all patients remain clinically well. CONCLUSIONS: Echocardiography imaging of young patients with exertional syncope or chest pain is reliable for the diagnosis of this coronary anomaly. It is surgically correctable; however, individual coronary anatomy may cause the surgical approach to vary. The postoperative outcome is excellent.

Relationship between changes in thyroid hormone level and severity of the postoperative course in neonates undergoing open-heart surgery.

BACKGROUND: Our aim was to determine whether the changes in thyroid function after open-heart surgery in neonates depend on the postoperative course. METHODS: Twenty neonates undergoing open-heart surgery for congenital heart disease were prospectively studied in the cardiac intensive care unit of a university-affiliated children's hospital. The patients were divided into two groups by level of inotropic support (high or mild). RESULTS: The groups were similar in age, bypass time and ultrafiltration volume. In both groups, there was a significant reduction in levels of thyroid-stimulating hormone and FT4 at 24 h postoperatively. However, in the high inotropic support group, FT4 was lower for a longer time. This group also had a significantly higher score on The Pediatric Risk of Mortality (PRISM; P < 0.042) and a longer duration of ventilation (P < 0.014). CONCLUSIONS: Neonates after open-heart surgery undergo changes in thyroid function characteristic of euthyroid sick syndrome. The degree of hypothyroxinemia may be related to the severity of illness and the postoperative course.

Optimizing early extubation after coronary surgery.

Early extubation after isolated coronary artery bypass surgery was assessed retrospectively in 545 of 779 patients treated by the same surgical team over one year. All underwent extubation within 10 hr of arrival at the cardiothoracic intensive care unit: 343 in < 6 hr and 202 in 6-10 hr. Operative mortality was 2.2%. Group comparisons revealed that patients who had earlier extubation were younger (61 vs. 66 years; p < 0.001), more likely to be male (72.5% vs. 61.3%; p < 0.05), with a shorter aortic crossclamp time (49.2 +/- 15.0 vs. 53.3 +/- 14.0 min; p < 0.05), cardiopulmonary bypass time (65 +/- 18.4 vs. 72.2 +/- 19.2 min; p < 0.05), intensive care unit stay (18.8 +/- 5.6 vs. 22.4 +/- 3.2 hr; p < 0.05) and postoperative hospital stay (5.2 +/- 2.2 vs. 6.0 +/- 2.4 days; p = 0.01). Extubation < 6 hr after cardiopulmonary bypass may accelerate recovery. The finding of no significant differences in clinical parameters between the groups suggests that efforts to further reduce the time to extubation might be worthwhile.

Repeat right ventricular outflow tract reconstruction using the Medtronic Freestyle porcine aortic root.

BACKGROUND AND AIM OF THE STUDY: A variety of valve substitutes are used for right ventricular outflow tract (RVOT) reconstruction in children and young adults after previous RVOT surgery that has led to significant pulmonary insufficiency and/or stenosis. Herein, the authors' experience with pulmonary valve replacement (PVR) using a porcine valved conduit late after previous RVOT surgery was reviewed. METHODS: Between August 2002 and April 2005, 31 patients (mean age 14.5 +/- 9.5 years; range: 1.2-33.1 years) underwent PVR using the Medtronic Freestyle porcine aortic root. These patients averaged two prior operations (range: 1-5) for the following diagnoses: tetralogy of Fallot +/- pulmonary atresia (n = 21); persistent truncus arteriosus (n = 5); aortic stenosis (Ross-Konno procedure) (n = 2); pulmonary atresia with intact ventricular septum; (n = 1); congenital pulmonary stenosis (n = 1); and transposition of the great arteries (n = 1). Of these patients, 29 (93.5%) underwent additional procedures at the time of pulmonary valve insertion including: branch pulmonary artery reconstruction (n = 21), atrial septal defect closure (n = 5), ascending aorta replacement (n = 4), pacemaker or defibrillator placement (n = 3), tricuspid valve repair (n = 2), ventricular septal defect closure (n = 2), and other procedures (n = 2). RESULTS: There were no early or late deaths. The mean hospital stay was 8.3 +/- 5.9 days (range: 4-25 days). One patient had a subarachnoid hemorrhage with transient left hemiparesis, and two patients had acute tubular necrosis with temporary dialysis treatment. All patients were well at a mean follow up of 13 +/- 9.3 months (range: 0.5-31 months). Echocardiography showed trivial or no pulmonary insufficiency in 30 patients (96.7%). The calculated mean peak systolic RVOT gradient by echocardiography was 23.4 +/- 7.6 mmHg. CONCLUSION: The Medtronic Freestyle bioprostheses demonstrated excellent short-term results for repeat RVOT reconstruction. This valve's hemodynamic characteristics are comparable to those of homografts, and it is an attractive alternative given the limited availability of homograft valves. A lack of availability in sizes smaller than 19 mm limits use of this valve in pediatric patients, and long-term results remain to be determined.

Anomalous hepatic venous drainage.

We present a 3-year-old boy born with anomalous hepatic venous drainage into the left atrium and a small sinus venosus atrial septum defect, in whom pulmonary arteriovenous malformations developed with progressive cyanosis. Surgical redirection of the hepatic venous drainage to the right atrium and closure of the atrial septal defect led to regression of the pulmonary arteriovenous malformations. However, in contrast to other reports, progressive pulmonary hypertension developed postoperatively.

Use of recombinant factor VII to control bleeding in a patient supported by right ventricular assist device after heart transplantation.

A 48-year-old man undergoing orthotopic heart transplantation for ischemic cardiomyopathy developed severe right heart failure. Severe intractable bleeding complicated implantation of a right ventricular assist device. Treatment with 9.6 mg of recombinant activated factor VII stopped the bleeding, and the patient could be transferred to the intensive care unit. This is the first reported case of the successful use of recombinant activated factor VII to control bleeding with subsequent right ventricular assist device function.

Recombinant factor VIIa (NovoSeven) as a hemostatic agent after surgery for congenital heart disease.

BACKGROUND: Postoperative bleeding and blood product requirements can be substantial in children undergoing open-heart surgery, and reexploration is required in 1% of cases. Recombinant activated factor VII (rFVIIa, NovoSeven, NovoNordisk, Denmark) is a hemostatic agent approved for the treatment of hemophilic patients with inhibitors to factor VIII or factor IX. It has also been used with success in other conditions. We present our experience with rFVIIa treatment for uncontrolled bleeding after open-heart surgery in five pediatric patients. METHODS: The study group consisted of five patients after open-heart surgery with excessive blood loss. The patients were treated with rFVIIa after failure of conventional treatment to control the bleeding. Blood loss, blood product consumption, and coagulation test results were recorded before and after rFVIIa administration. RESULTS: In all cases, blood loss decreased considerably after rFVIIa administration (mean 7.8 ml x kg(-1) x h(-1)), almost eliminating the need for additional blood products, and the prolonged prothrombin time normalized. In two patients with thrombocytopathy, rFVIIa helped to discriminate surgical bleeding from bleeding caused by a defect in hemostasis. No side effects of rFVIIa treatment were noted. CONCLUSIONS: These cases support the impression that RFVIIa is efficient and safe in correcting hemostasis in children after cardiopulmonary bypass when other means fail. However, the data are still limited, and more extensive research is needed.

Surgical management of aortopulmonary window and associated lesions.

BACKGROUND: Aortopulmonary window is a rare congenital heart defect commonly associated with other cardiac anomalies. Although single institutional experience is low, several surgical techniques have been reported. The purpose of this study is to describe our approach to the management of aortopulmonary window and its associated lesions. METHODS: Between February 1996 and November 2002, 13 patients underwent repair of aortopulmonary window. The age range went from 4 days to 5.5 months (mean 42 +/- 52 days), with 9 patients younger than 1 month old. The weight range was from 1.9 to 6.7 kg (mean 3.5 +/- 1.2 kg). Concomitant cardiac anomalies were present in 11 patients. The major additional anomalies were interruption of aortic arch in 4 patients and tracheal stenosis in 1 patient. Initial diagnoses were made using two-dimensional echocardiography only. RESULTS: There was one postoperative death. In general, patients with aortopulmonary window and additional major defects had a prolonged intensive care unit and hospital stay when compared with the other patients. Follow-up time ranged from 2 months to 6.8 years (mean of 2.5 +/- 2.2 years). There were no reoperations and no late deaths. Transcatheter balloon dilatation of the repaired aortic arch was required in 1 patient and of the right pulmonary artery in another. All other patients had good flow to both pulmonary arteries. No residual shunts were detected at the aortopulmonary window site, and pulmonary pressures were normal. CONCLUSIONS: Aortopulmonary window may be effectively diagnosed with echocardiography. Early surgical treatment (neonatal period, if possible) is safe and associated with the best long-term results, even in the presence of other cardiac anomalies. Complete separation and reconstruction of both aorta and pulmonary arteries under direct vision may prevent recurrence and distortion of adjacent structures.