Common arterial trunk in functionally univentricular hearts: a case series.

INTRODUCTION: The association of a univentricular heart defect with common arterial trunk is extremely rare. There is a lack of population-based outcome studies reported in the literature. METHODS: The hospital records, echocardiographic and other imaging modality data, outpatients' records, operation notes, and other electronic data were reviewed. Patients were reviewed, and the final outcomes of surgery were observed. RESULTS: Six cases (two males) with common arterial trunk presented over a 30-year period. Five had a complete unbalanced atrioventricular septal defect (83%) and one (17%) had tricuspid atresia associated with common arterial trunk. All had antenatal diagnosis. Two cases (33%) were excluded from initial surgical palliation due to Trisomy 21 in one and severe truncal valve regurgitation in one. Initial surgical palliation was performed in four cases (67%) at median age of 31 days (2-60) and consisted of disconnection and reconstruction of the pulmonary arteries and establishing controlled pulmonary blood flow. There were no early deaths. Conversion to cavopulmonary shunt was not possible in two due to severe airway problems in one and pulmonary arteries anatomy in one. They died at 11 and 16 months, respectively. Two patients (33%) underwent cavopulmonary shunt with 1 (17%) being alive at 18 months - 12 months after cavopulmonary shunt. The second patient proceeded to Fontan completion at 19 months but required catheter takedown 3 months later and died 3.5 years later. CONCLUSIONS: Univentricular hearts with common arterial trunk carry extremely poor short- to medium-term outcomes. This should inform antenatal and postnatal counselling and decision-making.

Pulmonary artery banding: still a role for staged bi-ventricular repair of intracardiac shunts?

OBJECTIVES: Although pulmonary artery banding remains a useful palliation in bi-ventricular shunting lesions, single-stage repair holds several advantages. We investigate outcomes of the former approach in high-risk patients. METHODS: Retrospective cohort study including all pulmonary artery banding procedures over 9 years, excluding single ventricle physiology and left ventricular training. RESULTS: Banding was performed in 125 patients at a median age of 41 days (2-294) and weight of 3.4 kg (1.8-7.32). Staged repair was undertaken for significant co-morbidity in 81 (64.8%) and anatomical complexity in 44 (35.2%). The median hospital stay was 14 days (interquartile range 8-33.5) and 14 patients (11.2%) required anatomical repair before discharge. Nine patients died during the initial admission (hospital mortality 7.2 %) and five following discharge (inter-stage mortality 4.8%). Of 105 banded patients who survived, 19 (18.1%) needed inter-stage re-admission and 18 (14.4%) required unplanned re-intervention. Full repair was performed in 93 (74.4%) at a median age of 13 months (3.1-49.9) and weight of 8.5 kg (3.08-16.8). Prior banding, 54% were below the 0.4th weight centile, but only 28% remained so at repair. Post-repair, 5/93 (5.4%) developed heart block requiring permanent pacemaker, and 11/93 (11.8%) required unplanned re-intervention. The post-repair mortality (including repairs during the initial admission) was 6/93 (6.5%), with overall mortality of the staged approach 13.6% (17/125). CONCLUSIONS: In a cohort with a high incidence of co-morbidity, pulmonary artery banding is associated with a significant risk of re-intervention and mortality. Weight gain improves after banding, but heart block, re-intervention, and mortality remain frequent following repair.

Ventricular fibromas in children, arrhythmia risk, and outcomes: A multicenter study.

BACKGROUND: Although ventricular fibromas are rare, they are the second most common type of cardiac tumor in children. While histologically benign, they have a propensity to cause malignant arrhythmias, with cardiac arrest often being the first presentation. OBJECTIVE: The purpose of this study was to evaluate the arrhythmia risk and management strategies for pediatric ventricular fibromas. METHODS: Fifteen centers in the British Paediatric Arrhythmia Group network were contacted to partake in the study to contribute cases. A detailed database search was performed at 2 hospitals for cases of ventricular fibromas. RESULTS: A total of 19 patients were included in the study. Arrhythmias were common, with 5 patients presenting with cardiac arrest and 5 others having documented ventricular tachycardia. Nine patients have undergone surgical resection at various hospitals, and all these patients have survived with good long-term outcomes. One patient who did not have any treatment died, presumably of a ventricular arrhythmia; another died of metastatic disease. There were no recurrences of arrhythmia after surgery, and the need for a defibrillator was alleviated in all cases. CONCLUSION: Ventricular fibromas have a high propensity to cause malignant arrhythmias, and if they are not managed appropriately, mortality is high. The outcomes of surgical resection are good, regardless of size, and this represents the best therapeutic option, with most patients being symptom free in the longer term.

Efficacy of Sentinel Lymph Node Biopsy in Detecting Axillary Metastasis in Breast Cancer Using Methylene Blue.

Breast cancer is the leading malignancy and the second leading cause of cancer-related deaths. Axillary lymph node status is a very important prognostic factor in breast cancer patients; nodal evaluation is therefore a critical part of breast cancer management. Axillary lymph node dissection results in significant morbidity. Sentinel lymph node biopsy (SLNB) is being used in many centers to stage the axilla in planning axillary dissection management of patients and hence plays an important part in reducing morbidity among patients with carcinoma breast. The objectives of this paper is to study the (1) efficacy of sentinel lymph node biopsy in detecting axillary metastasis, (2) location of sentinel lymph node in the axilla, (3) rate of involvement of sentinel lymph nodes, and (4) incidence of skip metastasis. Thirty-five patients with breast cancer with clinically node-negative axilla were selected for the study. Methylene blue dye was injected intralesional and perilesional 20 min prior to surgery. All patients underwent modified radical mastectomy with sentinel lymph node biopsy and axillary dissection and after pathological examination diagnostic statics, namely sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were computed. Sentinel lymph node was identified in all of these patients. Sixty percent patients had pathologically positive lymph nodes in the axilla. 90.48% patients of these had sentinel lymph nodes positive for malignant cells. Incidence of skip metastasis is 9.52%. 88.57% patients had sentinel lymph node mapped to level I lymph nodes. Sensitivity of SLNB is 90.48%, specificity is 85.71%, PPV of is 90.48%, NPV is 85.71%, and accuracy is 88.57%. Sentinel lymph node biopsy is an effective method of staging the axilla and deciding on axillary clearance in patients of carcinoma breast. Unnecessary axillary dissection and associated complications can be prevented in most of patients due to sentinel lymph node biopsy.

Impact of mode of failure and end-organ dysfunction on the survival of adult Fontan patients undergoing cardiac transplantation.

OBJECTIVES: Adults with failing Fontan circulation (FFC) represent a heterogeneous, high-risk group for cardiac transplantation with poor reported outcomes. We studied the impact of mode of Fontan failure (preserved versus impaired systolic ventricular function) and end-organ dysfunction on early survival in adults undergoing cardiac transplantation for FFC. METHODS: A single-centre, retrospective study of 26 adults (≥16 years) with FFC undergoing cardiac transplantation between 1990 and 2015. Patients were classified by the presence or absence of preserved systolic ventricular function (PVF). End-organ dysfunction was assessed by serum markers, including albumin, liver ultrasound and the presence of varices, ascites, splenomegaly and thrombocytopaenia (VAST score for portal hypertension). RESULTS: Thirty-day survival rate for the entire cohort was 69.2%, with 76.2% survival for the recent era. One-year Kaplan-Meier survival rate was 65.4%. Actuarial survival was poorer in those with PVF or heterotaxy (P = 0.01; log-rank test). Cox multivariable regression analysis confirmed PVF as an independent predictor for death (odds ratio, OR 5.38; confidence interval, CI 1.08-26.96; P = 0.04). In examining the PVF subset further, these patients had significantly higher VAST and liver ultrasound scores and lower serum albumin, compared with patients with impaired function. Patients with PVF and ≥moderate liver fibrosis on ultrasound or VAST score ≥2 accounted for two-thirds of the total mortality. CONCLUSIONS: Favourable cardiac transplantation outcomes can be achieved in adults with failing Fontan circulation. Patients with PVF may represent a distinct subset with more perturbed failing Fontan physiology and higher cardiac transplant mortality. We continue, however, to evolve and refine our strategies and are optimistic concerning future improvement in outcomes even in those with PVF.

Utility of echocardiography in identifying right ventricular extension of Wilm's tumor.

We present a rare case of a child with a Wilm's tumor with an intravascular tumor-thrombus extending from the inferior vena cava to the right ventricle via the tricuspid valve. Rapid tumor progression resulted in life-threatening clinical deterioration. Radiologic and cardiac imaging demonstrated the extent of the intravascular extension of her tumor-thrombus. Emergency neo-adjuvant chemotherapy resulted in rapid clinical improvement, so that complete surgical excision was possible. Following multimodality therapy, the child is now in remission. © 2014 Wiley Periodicals, Inc. J Clin Ultrasound 43:262-264, 2015.

Durable ventricular assist device support for failing systemic morphologic right ventricle: early results.

BACKGROUND: The systemic morphologic right ventricle (RV) in congenitally corrected transposition of the great arteries or after atrial switch for transposition of the great arteries is associated with late ventricular failure. Although the role of the left ventricular assist device (LVAD) in supporting the failing LV is established, the indications and outcomes of using LVAD in a systemic RV remain unclear. We assessed the role of a third-generation LVAD for systemic RV support. METHODS: Seven patients (mean age, 36 years) received the HeartWare (HeartWare International Inc, Framingham, MA) VAD for systemic RV failure (congenitally corrected transposition of the great arteries in 1 and after atrial switch in 6). Four patients (57%) had severe subpulmonic LV failure, and aggressive perioperative diuresis with or without hemofiltration was used to offload the subpulmonic LV. The indications of VAD were (1) bridge to transplant in 3 and (2) bridge to decision for a high transpulmonary gradient in 4. Transplantation outcome was compared with systemic RV failure without VAD bridge in 19 patients (years 1989 to 2013). RESULTS: Systemic RV support alone was achieved in all patients, with no early deaths (≤30 days). Overall, 6 (86%) returned home, 3 (44%) received a transplant, 2 (28%) died of noncardiac causes, and 2 (28%) continue on VAD support (median support, 232 days). Repeat catheterization (n = 4) showed an improved median transpulmonary gradient in 3 patients (median 18.5 mm Hg pre-VAD vs 8.0 mm Hg post-VAD). Two bridge-to-decision patients received transplants at 640 and 685 days. The stroke rate on VAD support was 43% (2 thromboembolic and 1 hemorrhagic; 3 with satisfactory recovery). De novo aortic regurgitation was 29% (n = 2; 1 valve replacement). All patients (n = 3) survived transplantation (vs 10.5% early mortality without VAD bridge; p = 1.00) and were well at follow-up (range, 53 to 700 days). CONCLUSIONS: The third-generation VAD provides durable support for systemic RV failure as a bridge to transplant and as a strategy to reduce pulmonary vascular resistance. Although concomitant subpulmonic LV failure is common, systemic RV support alone was achieved in all patients.

Is alternative cardiac surgery an option in adults with congenital heart disease referred for thoracic organ transplantation?

OBJECTIVES: We analysed the outcomes of adults with congenital heart disease (ACHD) referred for thoracic organ transplantation who underwent non-transplant cardiac surgery as an alternative management option. METHODS: Adult patients with congenital heart disease assessed for heart or heart-lung transplant were identified from the departmental database. A retrospective analysis of the medical records, transplant assessment data and surgical notes was carried out. RESULTS: One hundred and twenty-six patients were assessed between January 2000 and July 2011. Non-transplant cardiac surgery was performed in 14 (11%) patients. There were nine males with a median age of 37 years (range 21-42). The patients can be divided into four subgroups [left-sided lesions (n = 4), right-sided lesions (n = 3), systemic right ventricle (n = 5) and Fontan circulation (n = 2)]. Surgical procedures performed were: relief of systemic obstructive/regurgitant lesions ± endocardial fibroelastosis resection (n = 4, three pulmonary vascular resistance >6 Wood units), correction of right-sided regurgitant/stenotic lesions (n = 3), ventricular assist device for patients with a systemic right ventricle (n = 5) and re-fashioning of the Fontan pathway (n = 2). There were two early (5 and 30 days) and three late deaths (64, 232 and 374 days) with a 1-year mortality of 28%. None of the deaths occurred in patients with a two-ventricle circulation and atrio-ventricular concordance. Nine patients are alive at a median of 433 days (range 204-2456). The New York Heart Association class has improved in all survivors by at least one class at 3 and 6 months (P = 0.004 and 0.003). CONCLUSIONS: Alternative cardiac surgery can be undertaken in selected patients with ACHD referred for cardiopulmonary transplantation with a low mortality in patients with two ventricles and a systemic left ventricle. Ventricular assist devices carry a significant mortality in patients with a systemic right ventricle, although this offers a valuable palliation when there are no other options. The medium and long-term results are awaited.

Single-centre experience of 101 paediatric and adult Ross procedures: mid-term results.

We aimed to determine outcomes for the Ross procedure in paediatric and adult patients, with particular emphasis on survival, complication and reintervention rates. A retrospective review of 101 patients who had the Ross procedure in a congenital cardiac surgical centre serving a population of approximately 2.5 million was performed. There were 69 adults and 32 children with a mean age of 24.8 ± 13.9 years. Indications for surgery were aortic stenosis (48), regurgitation (10), mixed disease (35) and complex left outflow tract obstruction (8). The mean follow-up duration was 4.7 ± 3.7 years. The mini-inclusion technique was used to incorporate the autograft, and in all cases, pulmonary homografts were placed in the right ventricular outflow tract. Sub-aortic resection was also performed in six and Ross-Konno operations in eight patients. There were no early deaths and there was one late death secondary to endocarditis. Freedom from reintervention was 92% at 5 years and 77% at 10 years. Children were significantly more likely to require reintervention (16%, 5 of 32 versus 4%, 3 of 69, P = 0.05). The Ross procedure carries low early and mid-term mortality, and reintervention rates appear acceptable. The Ross procedure should be considered a feasible alternative to prosthetic valves in patients who require aortic valve replacement.

Cardiovascular abnormalities in Down's syndrome: spectrum, management and survival over 22 years.

BACKGROUND: The prevalence of cardiovascular anomalies in Down's syndrome is well described, but there are few data on spectrum, management and outcome. The authors aimed to provide this information for infants with Down's syndrome in a defined population over a 22-year period. METHODS: The regional paediatric cardiology database in Newcastle upon Tyne provided information on all cardiovascular anomalies, surgical treatment and outcome. Data was subdivided into two eras, 1985-1995 and 1996-2006, and surgical results and outcomes compared. Data on live births with Down's syndrome were obtained from the Northern Congenital Abnormality Survey (NorCAS). Denominator data on all live births in the region were obtained from UK Statistics. RESULTS: In 1985-2006 there were 754,486 live births in the population. 821 infants were live-born with Down's syndrome (1.09 per 1000 live births). 342 (42%) infants with Down's syndrome had a cardiovascular anomaly. The commonest anomaly was complete atrioventricular septal defect in 125 (37%) infants. Three patients had univentricular physiology. In 1985-1995, 101/163 (62%) infants had surgery with 30% mortality; in 1996-2006, 129/180 (72%) had surgery with 5% mortality. One patient underwent Fontan completion. There were two cardiac transplants for cardiomyopathy. One-year survival in Down's syndrome with a cardiovascular anomaly improved from 82% in 1985-1995 to 94% in 1996-2006. CONCLUSIONS: The incidence of cardiovascular anomalies in Down's syndrome was 42%. There has been a significant reduction in postoperative mortality and improvement in 1-year survival. Treatment modalities such as single ventricle palliation and cardiac transplantation are now considered in these patients.

Mortality in infants with cardiovascular malformations.

UNLABELLED: Cardiovascular malformations are an important cause of infant death and the major cause of death due to malformation. Our aims were to analyse and categorise all deaths in infants with cardiovascular malformations, and to analyse trends in mortality over time and influences on mortality. We obtained details of infant deaths and cardiovascular malformations from the population of one health region for 1987-2006. We categorised deaths by cause and by presence of additional chromosomal or genetic abnormalities or non-cardiac malformations. In 676,927 live births the total infant mortality was 4,402 (6.5 per 1,000). A total of 4,437 infants had cardiovascular malformations (6.6 per 1000) of whom 458 (10.3%) died before 1 year of age. Of this number, 151 (33%) deaths had non-cardiac causes, 128 (28%) were cardiac without surgery and 179 (39%) occurred from cardiac causes after surgery. Death was unrelated to the cardiovascular malformation in 57% of infants with an additional chromosomal or genetic abnormality, in 76% of infants with a major non-cardiac malformation and in 16% of infants with an isolated cardiovascular malformation. Terminations of pregnancies affected by cardiovascular malformations increased from 20 per 100,000 registered births in the first 5 years to 78 per 100,000 in the last 5 years. A total of 2,067 infants (47%) underwent surgery and of these 216 (10%) died before 1 year of age. CONCLUSIONS: A total of 10.4% of infants who died had a cardiovascular malformation and two-thirds of deaths were due to the malformation or its treatment. Mortality declined due to increasing termination of pregnancy and improved survival after operation.

Ablation of atrial tachyarrhythmias late after surgical repair of tetralogy of Fallot.

BACKGROUND: Patients with repaired tetralogy of Fallot may develop symptomatic and haemodynamic deterioration for many reasons such as arrhythmia, pulmonary regurgitation, and impairment in ventricular function. We describe a consecutive group of patients whose main clinical problem was atrial tachyarrhythmias. AIMS: To describe the clinical outcome of atrial tachyarrhythmias occurring late after surgical repair of tetralogy of Fallot; to define the circuits/foci responsible for these atrial tachyarrhythmias; to evaluate the outcome of computer-assisted mapping and catheter ablation in this patient group. METHODS AND RESULTS: Consecutive patients with surgically repaired tetralogy of Fallot and atrial tachyarrhythmias, who underwent catheter ablation between January, 2001 and June, 2007, were identified retrospectively from case records. Computer-assisted mapping was performed in all using either EnSite® (St Jude Medical Inc.) arrhythmia mapping and intra-cardiac catheter guidance system or CARTO™ (Biosense Webster Inc.) electroanatomical mapping systems. Ten patients (four males) with a median age of 39 plus or minus 8 years were studied. The total number of atrial tachyarrhythmias identified was 22 (six macro-reentrant, 16 micro-reentrant/focal). In nine patients, catheter ablation led to improvement in arrhythmia episodes and/or symptoms during follow-up of 41 plus or minus 20 months. Following ablation(s), five patients required pacing for pre-existing conduction disease and five needed further surgery for haemodynamic indications. All patients remained on anti-arrhythmic drugs. CONCLUSIONS: Patients with surgically repaired tetralogy of Fallot and atrial tachyarrhythmias typically have multiple arrhythmic circuits/foci arising from a scarred right atrium. Catheter ablation reduces arrhythmia frequency and improves symptoms. However, hybrid management is often required, comprising drugs, pacing, and further surgery tailored to the individual.

Fatal presentation of congenital isolated left ventricular apical hypoplasia.

Congenital isolated left ventricular apical hypoplasia has recently been recognised as a discrete clinical entity with well-defined diagnostic criteria on cardiac magnetic resonance imaging. This spectrum has been described in four previous cases, three of which presented with relatively mild symptoms and one with pulmonary oedema. All of these patients responded to standard medical management. We describe a sudden and fatal presentation of this anomaly in a previously well 19-year-old male, confirming the fact that this is not a benign condition but a spectrum with the potential for significant complications.

Enlarged bronchial collateral artery complicating recovery after arterial switch for simple transposition of the great arteries.

Enlarged bronchial collateral arteries can lead to significant left to right shunting resulting in pulmonary hypercirculation and hypoxemia and may complicate postoperative recovery after uneventful surgical repair. We describe a case of simple transposition of the great arteries in which the postoperative course was complicated by the presence of an enlarged bronchial collateral artery causing pulmonary oedema necessitating prolonged mechanical ventilatory support. Successful coil embolisation of the bronchial collateral resulted in resolution of the pulmonary oedema and successful extubation.

Prostaglandin therapy for ductal patency: how long is too long?

UNLABELLED: The management of neonates born with duct-dependent congenital heart defects in association with prematurity or low birth weight is challenging. We describe two preterm and low birth weight infants with duct-dependent congenital heart disease where cardiac surgery was successfully delayed by maintaining ductal patency using a prolonged prostaglandin infusion. This allowed time for growth and maturation and therefore reduced the risks associated with surgery and cardiopulmonary bypass. CONCLUSION: Maintenance of ductal patency by a prolonged prostaglandin infusion in low birth weight or preterm infants with duct-dependent congenital heart disease is a viable option that allows cardiac surgery to be delayed whilst awaiting further growth and development.

Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.

OBJECTIVE: Early failure of the Fontan-type circulation is a potentially fatal complication. We review our experience with cardiac transplantation in children presenting with end-stage heart failure in this scenario. METHODS: We performed a retrospective review. Between 1985 and 2003, 6 children aged less than 16 years were referred for cardiac transplantation. The indication for cardiac transplantation was end-stage cardiac failure early after the completion of the Fontan-type operation. RESULTS: All 6 patients listed for transplantation underwent cardiac transplantation; the median interval to transplantation from the operation was 36 days (range, 6-180 days). Four patients had undergone the Fontan procedure, and 2 had one-and-a-half-ventricle repair. All 6 patients were ventilated and inotrope dependent, with varying degrees of multiorgan dysfunction. One patient was bridged to transplantation with extracorporeal membrane oxygenation. The median age at transplantation was 7.1 years (range, 3-12.5 years), and weight was 18.9 kg (range, 11-35 kg). One patient died on the operating table (graft failure and hemorrhage). In 5 survivors the median intensive care unit stay was 10 days (range, 8-61 days). On follow-up of 6 to 81 months, there have been no subsequent deaths, and the quality of life in survivors is good. CONCLUSION: Rescue cardiac transplantation in the setting of an early failure of the Fontan-type circulation and end-stage cardiac failure is an effective treatment option and can be performed with acceptable early mortality and encouraging short-term to medium-term results.

Aarskog syndrome with aortic root dilatation and sub-valvular aortic stenosis: surgical management.

Aarskog syndrome is a familial condition associated with craniofacial anomalies, genital malformations and short stature. Affected children have significantly higher chance of having congenital heart disease (CHD) than the general population. We report the case of a child afflicted with progressive aortic root dilatation and sub-valvular aortic stenosis, successfully managed with aortic root and valve replacement. Given the association between Aarskog syndrome and CHD, cardiac surveillance should be undertaken in all affected children.