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We report a case of a 38-year-old female with an FLNA variant who underwent valve-sparing aortic root replacement. FLNA encodes Filamin A, an actin-binding protein. Our patient had aortic root dilation due to this variant. Aortic root repair was conducted using the David procedure, with modifications to account for tissue fragility associated with this genetic condition. This case demonstrates the value of patient-specific genetic information for the timing of surgery and operative course planning.

Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multitime point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology versus environmental response, and unique biological insights in each patient using a single platform measurement. Integration of a transcriptome workflow yielded unexpected insights into the complex interplay between host genetics and viral/bacterial specific mechanisms, highlighted by a unique case of virally induced genetics (VIG) within one of these 27 patients. The power of RNA-Seq to study unique patient biology while investigating environmental contributions can be a critical tool moving forward for translational sciences applied to precision medicine.

Surgical management of complex congenital heart disease (CHD) is challenging. Three-dimensional (3D) printing can improve multidisciplinary team decision-making, patient and family understanding, and education of medical professionals. We describe 3D printing for surgical management of five patients with complex CHD. The anatomical details of the 3D printed models were instrumental in planning surgical techniques especially in determining between single ventricle, 1.5 ventricle, and biventricular repair.

Left ventricular pseudoaneurysm (LV-PSA) is a rare complication following cardiac surgery, let alone in the pediatric population. Other known causes of LV-PSA are trauma, percutaneous cardiac intervention, and infections. This report describes the development of LV-PSA following surgical repair of ventricular septal defect (VSD) and coarctation of aorta (CoA) in an infant.

Extremely low birth weight neonates with complex congenital heart disease have increased mortality risk. Multi-organ dysfunction, pulmonary disease, fluctuating pulmonary vascular resistance, and complex cardiovascular anatomy create a challenge for effective management. We present the case of a 760-g neonate with dextro-transposition of the great arteries, ventricular septal defect, patent ductus arteriosus, and single coronary artery with proximal intramural segment of the right coronary artery branch. We describe features of preoperative care, surgical intervention, and postoperative course that enabled this infant to survive.

Precision medicine requires the translation of basic biological understanding to medical insights, mainly applied to characterization of each unique patient. In many clinical settings, this requires tools that can be broadly used to identify pathology and risks. Patients often present to the intensive care unit with broad phenotypes, including multiple organ dysfunction syndrome (MODS) resulting from infection, trauma, or other disease processes. Etiology and outcomes are unique to individuals, making it difficult to cohort patients with MODS, but presenting a prime target for testing/developing tools for precision medicine. Using multi-time point whole blood (cellular/acellular) total transcriptomics in 27 patients, we highlight the promise of simultaneously mapping viral/bacterial load, cell composition, tissue damage biomarkers, balance between syndromic biology vs. environmental response, and unique biological insights in each patient using a single platform measurement. Integration of a transcriptome workflow yielded unexpected insights into the complex interplay between host genetics and viral/bacterial specific mechanisms, highlighted by a unique case of virally induced genetics (VIG) within one of these 27 patients. The power of RNAseq to study unique patient biology while investigating environmental contributions can be a critical tool moving forward for translational sciences applied to precision medicine.One Sentence SummaryRNAseq shows the potential of a multidimensional workflow to define molecular signatures for precision/individualized medicine within the pediatric intensive care unit, identifying mechanisms such as viral-induced dominant genetics and infection signatures.

Three-dimensional (3D) printing is an emerging technology aiding diagnostics, education, and interventional, and surgical planning in congenital heart disease (CHD). Three-dimensional printing has been derived from computed tomography, cardiac magnetic resonance, and 3D echocardiography. However, individually the imaging modalities may not provide adequate visualization of complex CHD. The integration of the strengths of two or more imaging modalities has the potential to enhance visualization of cardiac pathomorphology. We describe the feasibility of hybrid 3D printing from two imaging modalities in a patient with congenitally corrected transposition of the great arteries (L-TGA). Hybrid 3D printing may be useful as an additional tool for cardiologists and cardiothoracic surgeons in planning interventions in children and adults with CHD.

Left main coronary artery atresia (LMCAA) is a rare congenital malformation with a nonspecific and varied clinical presentation. Ventricular dysfunction and mitral insufficiency are expected ischemic consequences in the neonatal period. Left internal mammary artery (LIMA) bypass grafting (CABG) is uncommon because of the technical difficulties in performing this procedure in neonates. We describe LMCAA revascularization with a LIMA graft and mitral valve repair in a 7-week-old neonate with successful outcome 1 year postoperatively.

The ductus arteriosus is a fetal vascular connection between the pulmonary and systemic circulations. It fails to close after birth in a small number of term infants, and in a larger number of infants with cyanotic congenital heart disease. In contemporary practice the majority of patients present with a patent ductus arteriosus (PDA) are premature infants before the gestational age of 28 weeks. The surgical management of PDA in preterm infants is critical for optimal outcomes and is discussed in this article.

The incidence of congenital heart defects is higher in infants with mutation of methylenetetrahydrofolate reductase ( MTHFR) gene. The MTHFR C677T gene decreases the bioavailability of folate and increases plasma homocysteine, a risk factor for thrombosis. There have been no reported cases in the literature on the clinical implications of this procoagulable state in the setting of cyanotic heart disease, which itself has prothrombotic predisposition. Two patients with hypoplastic left heart syndrome developed postoperative thrombotic complications, both were homozygous for MTHFR C677T. We present these cases and highlight the implications of MTHFR mutation in the management of complex congenital heart disease.

Tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is a rare congenital malformation. Although pulmonary artery (PA) anomalies have been observed in TOF, its association with disconnected PA is extremely rare. We report successful stenting of the disconnected left PA in a 3-year-old boy with TOF/APV followed by surgical reimplantation. The significance of this transcatheter intervention for guidance during surgery and the importance of visualizing a ductal stump on angiography as an indicator of disconnected PA are discussed.

OBJECTIVES: This study aimed to investigate the early and late outcomes of patients undergoing aortic valve replacement (AVR) with previous coronary artery bypass grafting (CABG) and patent grafts. METHODS: Between January 2000 and March 2010, 104 patients (87 males) with previous CABG ± concomitant surgery and patent grafts underwent AVR. The median age of the patients was 75 years (range: 37–90 years; inter-quartile range: 69–79 years) and the mean logistic EuroScore was 25.37 ± 16.8. The median time since the previous operation was 9 years (range 1–25; inter-quartile range: 7–14 years). The left internal mammary artery (LIMA) had been used in 75 patients (72.1%) and remained patent in 72 cases (96.0%). RESULTS: Thirty-day mortality was 7.7% (n= 8), which is less than the predicted mean logistic EuroScore. Isolated AVR was performed in 66 patients (63.5%). The LIMA was dissected and isolated (clamped or blocked with balloon) in 60 patients. The median hospital stay was 10 days (range: 4–183 days; inter-quartile range: 7–15.25 days). Nineteen patients (18.3%) had pulmonary complications, while 12 (11.5%) had acute kidney injury. Seven patients (6.7%) required permanent pacemaker. Six LIMAs (8.3%) were injured and repaired. Prolonged aortic cross-clamp (AXC) time (P= 0.038) and the presence of a previous LIMA graft (P= 0.045) were identified as independent predictors of 30-day mortality. The actuarial survival at 1 and 5 years was 89.4 ± 0.3 and 81.5 ± 0.5%, respectively. Perioperative intra-aortic balloon pump use (P= 0.036), prolonged AXC time (P= 0.004) and prolonged cardiopulmonary bypass time (P= 0.022) were associated with worse long-term overall survival on multivariate analysis. CONCLUSIONS: AVR post-CABG with patent grafts can be performed in high-risk patients with excellent short- and long-term outcomes and appears to be superior to published catheter-based interventions. In the absence of randomized trial data, we believe that open AVR remains the treatment of choice for aortic valve disease following prior CABG.

Hypervascular nodules occur commonly when there is hepatic venous outlet obstruction. Their nature and determinants in the Fontan circulation is poorly understood. We reviewed the records of 27 consecutive Fontan patients who had computerized tomography scan (CT) over a 4 year period for arterialised nodules and alterations in hepatic flow patterns during contrast enhanced CT scans and related these findings to cardiac characteristics. Mean patient age was 24 ± 5.8 years, (range 16.7–39.8) and mean Fontan duration was 16.8 ± 4.8 years (range 7.3–28.7). Twenty-two patients demonstrated a reticular pattern of enhancement, 4 a zonal pattern and only 1 demonstrated normal enhancement pattern. Seven (26%) patients had a median of 4 (range 1–22) arterialised nodules, mean size 1.8 cm (range 0.5 to 3.2 cm). All nodules were located in the liver periphery, their outer aspect lying within 2 cm of the liver margin. Patients with nodules had higher mean RA pressures (18 mmHg ± 5.6 vs. 13 mmHg ± 4, p = 0.025), whereas their mixed venous saturation and aortic saturation was not significantly different (70% ± 11 vs. 67% ± 9 and 92% ± 10 vs. 94% ± 4, p > 0.05). Post-mortem histology suggests focal nodular hyperplasia is the underlying pathology. ConclusionsAbnormalities of hepatic blood flow and the presence of arterialised nodules are common in the failing Fontan circulation. They occur especially when central venous pressures are high, and very likely indicate arterialisation of hepatic blood flow and reciprocal portal venous deprivation. The underlying pathology is most likely focal nodular hyperplasia.

Background: The aging of the population has resulted in an increasing number of elderly patients undergoing cardiac operations. We reviewed our experience in patients over the age of 80 undergoing primary aortic valve replacement (AVR) with or without CABG. Methods: Between 2000 and 2008, 345 patients (226 male) ≥80 years underwent primary AVR in our unit. The notes of these patients were retrospectively reviewed and follow-up information was obtained from their general practitioners. They had a mean age of 82.9 ± 2.3 years and a median logistic EuroSCORE of 13.4 (IQR 9.4, 19.1). Isolated AVR was performed in 161 patients (45.5%), and 184 (51.6%) patients underwent combined AVR and CABG. A quality of life questionnaire was sent to all survivors. Results: Hospital mortality occurred in 17 patients (4.9%), which was significantly lower than the mortality predicted by logistic EuroSCORE (16.2%, p < 0.01). Hospital mortality was comparable between patients undergoing isolated AVR and those undergoing additional CABG (4.3% vs. 5.4%, respectively). Actuarial survival at one and five years was 90.1 ± 1.6% and 77.2 ± 2.9%, respectively. There was a 62% response on the questionnaire showing 70% of the patients were NYHA I and 83.7% were satisfied with the operation outcome. Conclusions: AVR can be undertaken with excellent results in octogenarians and the current risk is significantly lower than what is predicted with conventional risk-scoring systems. Patients with advanced age should not necessarily be excluded from being candidates for AVR. (J Card Surg 2011;26:466-471)

Background The purpose of this study was to analyze the factors affecting reoperation after primary biventricular atrioventricular septal defect (AVSD) repair. Methods Between April 1997 and April 2007, 93 consecutive patients underwent surgery for biventricular correction of AVSD with a median age of 5.8 months (range, 9 days to 68.9 years). Fifty-three patients had complete AVSD, 6 patients had an intermediate type, and 29 patients had partial AVSD; 4 patients had a complete AVSD with associated tetralogy of Fallot, and 1 patient had a complete AVSD with double-outlet right ventricle. Results There was no in-hospital mortality. There were 2 late deaths (2.2%). Forty-three reoperations were performed in 23 patients (24.7%), of which 18 were for repair of significant left atrioventricular valve regurgitation and 8 were mitral valve replacements. Seven patients (7.5%) required insertion of a permanent pacemaker. The overall 5-year freedom from reoperation after AVSD repair was 73.6% ± 4.8%. In the multivariate analysis for complete AVSDs, Down syndrome ( p = 0.01) and the presence of right ventricular dominance ( p = 0.03) were independent predictors of reoperation. At last follow-up, 76 patients (83.5%) were in New York Heart Association class I, and 68 patients (74.7%) were not taking any heart failure medications. Echocardiographic examination showed absent to mild left atrioventricular valve regurgitation in 76.5%; moderate, in 19.8%; and severe, in 3.7% of patients. Conclusions Down syndrome and right ventricular dominance are independent predictors of reoperation after complete AVSD repair. Biventricular repair of isolated AVSD with a small left ventricle can be successfully accomplished with no mortality.

Due to their stability and rapid physiologic response, circulating microRNAs (miRNAs) are promising biomarkers. However, little is known about the extent to which circulating miRNA levels are reflective of homeostasis of specific tissues. Plasma was collected from 10 patients aged 3 months to 4

Background: The Biovalsalva aortic root prosthesis incorporates an Elan porcine stentless biological aortic valve suspended within a triple-layered vascular conduit with preformed aortic sinuses of Valsalva. This study compared implantation of the Biovalsalva prosthesis with a “handsewn” composite bioprosthetic graft (CE Perimount bovine bioprosthesis anastomosed to a gelatin-impregnated gelweave Dacron graft). Methods: Between December 2004 and January 2009, 39 patients underwent elective or urgent aortic root replacement (modified Bentall procedure with coronary button reimplantation) using a Biovalsalva (n = 21) or a handsewn bioprosthesis (n = 18) for aortic root dilatation. Results: There was no significant difference in the preoperative variables between the two study groups including age (70.7 ± 1.7 vs. 67.6 ± 2.9 years, p > 0.05). There was no in-hospital mortality. Three patients in each group underwent concomitant aortic hemi-arch replacement. Patients who underwent Biovalsalva implantation had a reduced need for transfusion of blood (1.25 ± 0.32 vs. 3.17 ± 0.71 units, p < 0.05) and fresh frozen plasma (2.78 ± 0.39 vs. 1.85 ± 0.31, p < 0.05), and reduced mediastinal blood loss (416 ± 52 vs. 583 ± 74 mL, p < 0.05) compared to those with a handsewn bioprosthesis. Cardiopulmonary bypass time (141 ± 6 vs. 170 ± 17 minutes, p = NS) and aortic cross-clamp time (113 ± 6 vs. 115 ± 7 minutes, p = NS) were similar. Postoperative echocardiography demonstrated excellent hemodynamic function of the Biovalsalva prosthesis (mean size 25.1 ± 0.4 mm valved conduit) with a peak pressure gradient of 26.2 ± 1.9 mmHg and no or trivial valvular regurgitation. Conclusions: The Biovalsalva prosthesis should be considered for patients requiring a biological aortic root replacement. It offers an “off-the-shelf” preassembled composite biological valve conduit with excellent hemostatic and hemodynamic properties. (J Card Surg 2010;25:321-326)

Background Three-dimensional echocardiography offers new insights into valvar function in atrioventricular septal defects (AVSDs). The aim of this study was to identify a morphological marker to predict the functional outcomes of left atrioventricular valves (AVVs) following the repair of AVSDs. Methods Twenty-nine consecutive patients were evaluated preoperatively using 2-dimensional and 3-dimensional echocardiography. The angle of the AVV relative to the crux of the heart was measured in multiplanar review mode. Results The severity of postoperative left AVV regurgitation was correlated with preoperative valvar angle, being more acute in patients with moderate or severe regurgitation (mean, 57 ± 13° vs 83 ± 9° in patients with no or mild regurgitation; P = .002). Angles ≤ 59° predicted severe regurgitation with 79% specificity. Conclusions Multiplanar review of 3-dimensional data sets is valuable for the assessment of the functional morphology of AVSD valves. Using this technique, more acute AVV angles predicted increased likelihood of severe regurgitation following surgical repair.

Background: The failing Fontan circulation is associated with hepatic impairment. The nature of this liver injury is poorly defined. Objective: To establish the gross and histological liver changes of patients with Fontan circulation relative to clinical, biochemical and haemodynamic findings. Methods: Patients were retrospectively assessed for extracardiac Fontan conversion between September 2003 and June 2005, according to an established clinical protocol. Twelve patients, mean age 24.6 (range 15.8–43.4) years were identified. The mean duration since the initial Fontan procedure was 14.1 (range 6.9–26.4) years. Results: Zonal enhancement of the liver (4/12) on CT was more common in patients with lower hepatic vein pressures (p = 0.007), and in those with absent cardiac cirrhosis on histological examination (p = 0.033). Gastro-oesophageal varices (4/12) were more common in patients with higher hepatic vein pressure (21 (6.3) vs 12.2 (2.2) mm Hg, p = 0.013) and associated with more advanced cirrhosis (p = 0.037). The extent of cirrhosis (7/12) was positively correlated with the hepatic vein pressure (r = 0.83, p = 0.003). A significant positive correlation was found between the Fontan duration and the degree of hepatic fibrosis (r = 0.75, p = 0.013), as well as presence of broad scars (r = 0.71, p = 0.021). Protein-losing enteropathy (5/12) occurred more frequently in patients with longer Fontan duration (11.7 (3.2) vs 17.9 (6.1) years, p = 0.038). Conclusions: Liver injury, which can be extensive in this patient group, is related to Fontan duration and hepatic vein pressures. CT scan assists non-invasive assessment. Cardiac cirrhosis with the risk of developing gastro-oesophageal varices and regenerative liver nodules, a precursor to hepatocellular carcinoma, is common in this patient group.

Pregnancy is typically not recommended in patients with Fontan circulation. Although patients are well aware of the risks for the mother and fetus, an increasing number opt to become pregnant. The higher rate of survival into adulthood post-Fontan procedure is a likely factor as a result of improved management of single ventricle anatomy. Postpartum thromboembolism is a known complication, but its prevalence and management are not clearly defined. We present a case of massive pulmonary embolism two weeks postpartum in a patient with double inlet left ventricle palliated with lateral tunnel Fontan.

Fontan palliation is used when biventricular repair (BVR) is not possible. Early outcomes are acceptable; however, the long-term sequelae include protein-losing enteropathy, declining functional status, increased pulmonary vascular resistance, heart failure, and hepatic and renal dysfunction. These adverse events are characteristic of persistent venous hypertension and may be avoided if restoring biventricular circulation is possible. Arrhythmias are a common adverse event, particularly in patients with an atriopulmonary connection, which may lead to acute decompensation and early death. We describe a 30-year-old woman who underwent successful BVR for pulmonary atresia with intact ventricular septum and demonstrate that where favorable anatomy exists with a failing Fontan, BVR should be considered.

ObjectiveMultiplanar re-formatting of full-volume three-dimensional echocardiography data sets offers new insights into the morphology of atrioventricular septal defects. We hypothesised that distortion of the alignment between the atrial and ventricular septums results in imbalanced venous return to the ventricles, with consequent proportional ventricular hypoplasia.MethodsA single observer evaluated 31 patients, with a mean age of 52.09 months, standard deviation of 55, and with a range from 2 to 264 months, with atrioventricular septal defects, of whom 17 were boys. Ventricular imbalance, observed in nine patients, was determined by two-dimensional assessment, and confirmed at surgical inspection in selected cases when a univentricular strategy was undertaken. Offline analysis using multiplanar re-formatting was performed. A line was drawn though the length of the ventricular septum and a second line along the plane of the atrial septum, taking the angle between these two lines as the atrioventricular septal angle. We compared the angle between 22 patients with adequately sized ventricles, and those with ventricular imbalance undergoing univentricular repair.ResultsIn the 22 patients undergoing biventricular repair, the septal angle was 0 in 14 patients; the other eight patients having angles ranging from 1 to 36, with a mean angle of 7.4°, and standard deviation of 11.1°.The mean angle in the nine patients with ventricle imbalance was 28.6°, with a standard deviation of 3.04°, and with a range from 26 to 35°. Of those undergoing univentricular repair, two patients died, with angles of 26 and 30°, respectively.ConclusionsThe atrioventricular septal angle derived via multiplanar formatting gives important information regarding the degree of ventricular hypoplasia and imbalance. When this angle is above 25°, patients are likely to have ventricular imbalance requiring univentricular repair.

The effect of leukocyte-depleting filters on the total and activated leukocyte counts and the expression of surface adhesion molecules CD11b, CD18, and CD62L during the in vitro extracorporeal circulation of human blood was studied. A 200 ml blood sample was taken from 10 patients undergoing CABG surgery. The blood was circulated for 60 minutes within an experimental extracorporeal circuit. A leukocyte-depleting filter was attached in five circuits (filtered group). In five other circuits, no filter was used (controls). Total leukocyte counts were determined manually. Activated leukocytes were identified using nitroblue tetrazolium staining. The expression of CD11b, CD18, and CD62L was measured with flow cytometry. At 60 minutes, total leukocyte counts were reduced by 49% from the baseline values in the filtered group and 10% in the control group (p < 0.0001). Activated leukocyte counts decreased by 86% in the filtered group and increased by 116% in the control group (p < 0.0001). In the filtered group, the expression of CD11b, CD18, and CD612L decreased by 60%, 21%, and 79%, respectively, and in the control group it increased by 24%, 6%, and 28% (p < 0.0001). Leukocyte-depleting filters preferentially remove activated leukocytes and reduce the expression of CD11b, CD18, and CD62L during the in vitro extracorporeal circulation of human blood.

The authors examined the effect of blood temperature within the cardiopulmonary bypass (CPB) circuit on the efficacy of an arterial line filter. Eighty patients undergoing elective, primary coronary artery bypass grafting under CPB were prospectively randomized in two equal groups. Blood temperature was kept at 35 degrees C in the first group and reduced to 28 degrees C in the second group. Twenty patients in each group had an arterial line LG6 filter attached onto CPB circuit. The other 20 patients in each group (controls) had a non-leukocyte-depleting filter. Blood samples (10 ml) were taken before CPB, at 5 minutes on CPB, at 30 minutes on CPB, 5 minutes after aortic clamp removal, and 6 hours postoperatively. Leucocytes were counted under light microscopy. Activated leucocytes were identified using nitroblue tetrazolium staining. Patients undergoing leucodepletion had significantly lower total and activated leukocyte counts than control patients in both groups (p0.05). Patients having a leukocyte-depleting filter at a CPB temperature of 35 degrees C had significantly lower total leukocyte counts (p0.05) than those having a leukocyte-depleting filter at a CPB temperature of 28 degrees C (p0.05). However, there were not statistically significant differences in the activated leukocyte counts between the two leucodepleted groups (p0.05). This study shows that warm blood temperature within the CPB circuit has a positive effect on the overall leucodepleting efficacy of the LG6 filter. Activated leucocytes, however, seem to be depleted at similar rates irrespective of the blood temperature in the CPB circuit.

Background Cardiopulmonary bypass is associated with a whole body inflammatory reaction. Exhaled nitric oxide increases in inflammatory lung conditions (eg, asthma) in proportion to the severity of inflammation, and has been proposed as a marker of pulmonary inflammation during cardiopulmonary bypass. This study evaluated the effect of arterial line leukocyte depletion during cardiopulmonary bypass on the rate of alveolar production of exhaled nitric oxide. Methods One hundred and ten patients with normal respiratory function, undergoing first time coronary artery bypass grafting, were randomized to two groups. Fifty-five patients had an arterial line leukocyte-depleting filter and 55 controls had a standard arterial line filter. Nitric oxide was sampled through an endotracheal Teflon tube after median sternotomy, but before cardiopulmonary bypass and 30 minutes after cardiopulmonary bypass, using a real time chemiluminescence analyzer, during the phase of the alveolar plateau. Results There were no significant differences in the precardiopulmonary bypass values of exhaled nitric oxide between the control (2.92 ± 1.51 ppb/s) and the leukodepletion group (3.11 ± 1.53 ppb/s) ( p = 0.4). After cardiopulmonary bypass, the rate of alveolar production of exhaled nitric oxide increased in both groups, being, however, significantly higher in the control group (4.68 ± 1.89 vs 3.72 ± 1.33 ppb/s) ( p = 0.02). Conclusions Continuous arterial line leukocyte-depletion significantly reduces the rate of alveolar production of exhaled nitric oxide after cardiopulmonary bypass. Changes in the rate of alveolar production of exhaled nitric oxide may be used as a marker of pulmonary inflammation in coronary artery surgery.

ObjectivesSymptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery.MethodsBetween 1997 and 2002, 15 patients (mean age, 19.7 ± 7.0 years) underwent conversion operations 12.7 ± 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation.ResultsNo mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 ± 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 ± 7.58 days). At follow-up (mean, 42.6 ± 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 ± 4.01 minutes to 10.45 ± 2.11 minutes (P < .05). Need for antiarrhythmic agents decreased postoperatively (patients receiving ≤1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P < .05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage.ConclusionsFontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.

The management of cardiac leiomyosarcoma is still controversial. Due to the rare occurrence and late presentation of such tumours the treatment is essentially palliative. We report on a case in a young female who presented with catastrophic haemodynamic instability and pulmonary venous obstruction by a large mass in her left atrium. Right pneumonectomy was performed as part of emergency surgery in this patient with a left atrial tumour of unknown histology. We feel that an attempt at complete clearance of these tumours is justified, as surgery remains the only chance of long-term survival. Postoperatively, adjuvant chemotherapy and/or radiotherapy may have a role.

Primary cardiac tumors are very rare, with a reported incidence of 0.15% to 0.2% in autopsy series. They can be life threatening because of myocardial compression and ventricular dysfunction. Once diagnosed during pregnancy, the clinical condition of the baby is monitored because of the risk of rupture of the tumor capsule. The authors report a rare case of a neonate who presented with respiratory and cardiac compromise due to cardiac tamponade necessitating emergency exploration of the pericardium and excision of tumor. A well-encapsulated tumor measuring around 5 cm and bigger than the heart was completely excised. This was diagnosed to be an immature teratoma. Follow-up echocardiogram was normal, and on serial monitoring, alpha-fetoprotein was within normal limits. The baby was discharged home with no complications. Intrapericardial teratoma in neonates is a surgical emergency if presented with significant pericardial effusion. It can be a challenge if diagnosed in utero with rupture before the viability of pregnancy. A multidisciplinary team approach is necessary to manage such situations. Complete excision is necessary because of its association with tissues of malignant potential.

Objective: The most appropriate management of aortic stenosis (AS) in children remains controversial. The purpose of this study was to determine the outcome following open valvotomy for AS in children. Methods: Ninety-seven consecutive, unselected, children (mean age 3.2 ^ 3.6 years, 1 day‐15 years) underwent an open valvotomy for criticalOna 36U or severeOna 61U AS between 1979 and 2000 in Southampton. Twenty-six were neonates (1‐31 days), 27 were infants (1‐12 months) and 44 were older children (1‐15 years). Mean followup was 10 ^ 5.4 years, 1 month‐21.9 years. Results: Two neonates died early giving an overall operative mortality of 2.1% (7.7% for the neonates and 0% for infants and older children). The mean aortic gradient was reduced from 76 to 24.5 mmHgOP , 0:0001U: Residual or recurrent AS occurred in 17 patients and severe aortic regurgitation in eight patients. Kaplan‐Meier 10-year freedom from residual or recurrent AS was 83.1 ^ 4.7% and from severe aortic regurgitation was 95.3 ^ 2.7%. Twenty-five patients required an aortic re-operation or re-intervention, 18 of whom had an aortic valve replacement (AVR) (mean valve size 21.8 ^ 0.9 mm, range 21‐25 mm). Ten-year freedom from any aortic re-operation or re-intervention was 78.4 ^ 5.2% and from AVR was 85.1 ^ 4.6%. There were ten late deaths. Overall 10year survival, including hospital mortality, was 90.2 ^ 3.1% (69.7 ^ 9.7% for the neonates, 92 ^ 5.4% for the infants and 100% for older children,OP , 0:0001U: Ten-year survival for children with isolated ASOna 69U was 100% and for those with associated cardiovascular problemsOna 28U was 67.3 ^ 8.9%OP , 0:0001U: All survivors are in New York Heart Association functional class I. Conclusions: Open valvotomy remains the gold standard in the management of AS in neonates, infants and older children. It is associated with low operative mortality and provides lengthy freedom from recurrent AS and regurgitation. Re-operations are common but if AVR is required, implantation of an adult-sized prosthesis is usually possible. There is a late death-hazard for those with severe associated lesions, but the survival prospects for the patients with isolated AS are excellent. q 2001 Elsevier Science B.V. All rights reserved.

Introduction: 10% of blood issued by the National Blood Service (220 000) is utilised in cardiac procedures. Transfusion reactions, infection risk and cost should stimulate us to decrease this transfusion rate. We tested the efficacy of autotransfusion of washed postoperative mediastinal fluid in a prospective randomized trial. Patients and methods: 166 patients undergoing coronary artery bypass grafting (CABG), valve or CABG+valve procedures were randomized into three groups. The indication for transfusion was a postoperative haemoglobin (Hb) Results: There was no significant difference in age, sex, type of operation, CPB time and preoperative Hb and PCV between the groups. Blood requirements were as shown. Group A (n=54) Group B (n=58) Group C (n=54) P Bank blood (pts) 28 30 46 0.0007 Bank blood ml/pt 298±49 343±52 508±49 0.00 Total blood ml/pt 404±50 347±51 508±50 0.03 Mediastinal drain ml/pt 652±51 624±48 686±50 NS Day 1 Hb g/dl 11.2±0.51 10.9±0.13 10.6±0.13 0.002 Twelve patients in group A and 10 in group B did not require a homologous transfusion following processing of the mediastinal drainage fluid. Conclusion: Autotransfusion of washed postoperative mediastinal fluid can decrease the amount of homologous blood transfused following cardiac surgery. There was no demonstrable benefit in processing blood from the CPB circuit as well as mediastinal drainage fluid.

Background —Atrial fibrillation (AF), a cardiac arrhythmia arising from atrial re-entrant circuits, is a common complication after cardiac surgery, but the proarrhythmic substrate underlying the development of postoperative AF remains unclear. This study investigated the hypothesis that altered expression of connexins, the component proteins of gap junctions, is a determinant of a predisposition to AF. Methods and Results —The expression of the 3 atrial connexins–connexins 43, 40, and 45—was analyzed at the mRNA and protein levels by Northern and Western blotting techniques and immunoconfocal microscopy in right atrial appendages from patients with ischemic heart disease who were undergoing coronary artery bypass surgery. Twenty percent of the patients subsequently developed AF, which allowed retrospective division of the samples into 2 groups, non-AF and AF. Connexin43 and connexin45 transcript and protein levels did not differ between the groups. However, connexin40 transcript and protein were expressed at significantly higher levels in the AF group. Connexin40 protein was markedly heterogeneous in distribution. Conclusions —Atrial myocardium susceptible to AF is distinguished from its nonsusceptible counterpart by elevated connexin40 expression. The heterogeneity of connexin distribution could give rise to different resistive properties and conduction velocities in spatially adjacent regions of tissue, which become enhanced and, hence, proarrhythmic the higher the overall level of connexin40.

The mouse is currently widely used as a model organism in the analysis of gene function but how developmentally regulated patterns of connexin gene expression in the mouse compare with those in the human is unclear. Here we compare the patterns of connexin expression in the heart during the development of the mouse (from embryonic day 12.5 to 6 weeks postpartum) and the human (at 9 weeks gestation and adult stage). The extent of connexin43 expression in the ventricles progressively increased during development of the mouse heart. The developmental pattern of expression for connexins 40 and 45 in the mouse heart was similar, but not identical, and in the ventricles showed a progressive and preferential expression in the conduction system. In general, these dynamic changes of connexins 43, 40 and 45 during mouse cardiac development appear to be mirrored in the human.

Anomalous origin of the right pulmonary artery from the ascending aorta, also called hemi-truncus arteriosus, is a rare congenital cardiovascular malformation. Around 95% of the reported cases with hemi-truncus arteriosus present in infancy. We describe a case of a 41-year-old female presenting with a long-standing history of intermittent haemoptysis. Cardiac catheterisation confirmed the diagnosis of hemi-truncus arteriosus. The definitive treatment of hemi-truncus arteriosus in adulthood is an extremely high-risk procedure. We decided to perform palliative right pulmonary artery banding which represents a simple but effective procedure for the management of this complex condition.

Operative mortality of inferior postinfarct ventricular septal rupture (VSR) remains high. Previous reports have described a transatrial approach to access the VSR avoiding a ventriculotomy, thereby reducing the potential risks of myocardial damage, hemorrhage, and ventricular arrhythmias. The transatrial right ventricular patch or direct suture repair, however, is exposed to the left-to-right pressure gradient and is at risk of residual shunting. In this report, a transatrial approach is used, with direct suture of the VSR, patch exclusion of the right ventricle, and biological glue instillation to protect the patch from left ventricular pressures.

Abstract —Upregulation of connexin43-gap junctions is associated with transition of contractile vascular smooth muscle cells (SMCs) to the synthetic state. To determine whether phenotypically distinct subpopulations of medial SMCs differentially express connexin43, we investigated the human distal internal mammary artery, a structurally heterogeneous vessel with features ranging from elastic to elastomuscular to muscular. Immunoconfocal microscopy combined with quantitative analysis and complemented by in situ hybridization showed that SMCs in the elastic medial regions expressed high levels of connexin43 but low levels of desmin, whereas those of muscular medial regions expressed low levels of connexin43 but high levels of desmin. Ultrastructurally, SMCs of both regions were of the contractile phenotype, but the former cells were irregular in shape with relatively prominent synthetic organelles whereas the latter were spindle shaped with fewer synthetic organelles. Vimentin, smooth muscle α-actin, calponin, h-caldesmon, and myosin heavy chains (SM1 and SM2) were equally highly expressed by most cells in both subpopulations. The connexin43/desmin expression pattern of SMCs in regions of intimal thickening resembled those of elastic medial regions. These findings refine the view suggested from previous studies that high levels of connexin43 expression are associated with SMCs of a less contractile/more synthetic phenotype. In the internal mammary artery, the 2 subpopulations of SMCs with markedly different connexin43 expression levels both represent a differentiated contractile phenotype, but the subpopulation showing high levels of connexin43-gap junctions is characterized by low levels of desmin and structural features that reflect a more synthetic tendency.

Objectives: The National Blood Service issues 2.2 million units of blood per year, 10% of these (220 000) are utilized in cardiac procedures. Transfusion reactions, infection risk and cost should stimulate us to decrease this transfusion rate. We test the efficacy of autotransfusion following surgery in a prospective randomized trial. Methods: One hundred and twelve patients undergoing CABG, valve or CABG 1 valve procedures were randomized into two groups. Group A received washed postoperative drainage fluid and group C were controls. The indication for transfusion was a postoperative haemoglobin (Hb) , 10 g/l or a PCV , 30. There was no significant difference in preoperative and operative variables between the groups. Results: Twenty-eight patients in group A and 46 in group C required homologous transfusion (Pa 0:0008). Group A patients required 298 ^ 49 ml of banked blood per patient, group C 508 ^ 49 ml (Pa 0:003). There was no difference in total blood required (volume autotransfused 1 volume banked blood transfused) between the groups (group A 404 ^ 50 ml, group C 508 ^ 50 ml) or in mean total mediastinal fluid drainage (group A 652 ^ 51 ml, group C 686 ^ 50ml). The mean Hb concentration was significantly higher in group A on day 1 (11: 2g =dl ^ 51 vs:10: 6g =dl ^ 13 (Pa 0:002)). No morbidity was associated with autotransfusion. Conclusion: Autotransfusion can decrease the amount of homologous blood transfused following cardiac surgery. This represents a benefit to the patient and a decrease in cost to the health service. q 1999 Elsevier Science B.V. All rights reserved.

Objective: The value of coronary artery bypass grafting (CABG) at the time of repair of a post-infarct ventricular septal defect (VSD) remains controversial. The aim of this study was to analyse the effect of CABG on early mortality and survival following repair of an acquired VSD. Methods: Over 23 years, 179 patients, 118 male, 61 female, mean age 66 years (range 43‐80), have undergone repair of a post-related VSD in our unit. A total of 29 patients, who predominantly form the earlier part of the series, were operated on greater than 1 month after the infarct and are, therefore, excluded. Coronary angiography was performed in 98 (65.3%) of the remaining 150 patients. Of these, 41 had coronary artery disease (CAD) limited to the infarct-related vessel and 57 had additional significant CAD. Those with CAD limited to the infarct-related vessel were not grafted (Group A). Of those, 40 with significant CAD underwent CABG at the time of VSD repair (Group B) and 17 did not (Group C). In 52 patients the coronary anatomy was not documented (Group D). Risk factors for early mortality were evaluated using logistic regression. Actuarial survival was compared using log rank and Wilcoxon tests. Cox’s proportional hazards method was used to determine factors affecting survival. Results: Overall, 30 day mortality was 32%. CABG did not significantly decrease operative mortality (logistic regression). There was no statistically significant difference in early mortality or actuarial survival between the four groups. CABG was not associated with an increased survival (Cox’s method). Conclusions: Concomitant CABG at the time of VSD repair does not affect early mortality nor confer survival benefits. There seems to be no demonstrable benefit in revascularisation at the time of repair and, therefore, it may be unnecessary to perform CABG or coronary angiography in these patients. © 1998 Elsevier Science B.V. All rights reserved.

The study assesses the impact of a program using a model of knowledge transfer on the long-term development of a pediatric cardiac service in Lithuania. A team from the United Kingdom evaluated Lithuanian pediatric cardiac services and provided support in areas targeted for improvement. The total number of infant operations performed in Lithuania from 1990 to 2008, together with in-hospital mortality rates, was broken down into 3 time periods and analyzed to estimate the efficacy of the program: (1) 1990-1998, before the program; (2) 1999-2002, during the program; (3) 2003-2008, following the end of the program. Lithuanian results in children older than 1 year were comparable with other European centers. However, only 315 infant and neonatal cardiac procedures were performed between 1990 and 1998, and there was an in-hospital mortality of 34.2%. Between 1999 and 2002, the UK team performed 23 highly complex demonstration procedures (in-hospital mortality, 13%). During the same period the Lithuanian team performed 305 additional operations in neonates and infants, and in-hospital mortality decreased to 18.7%. From 2003 to 2008 results continued to improve—559 infant operations were performed, with in-hospital mortality of 11.3% ( P < .0001). Knowledge transfer has led to substantial and sustainable long-term improvement in the results of infant cardiac surgery in Lithuania. Demonstrating techniques and care on a limited number of more complex cases is an efficient way of transferring knowledge and skills to the developing pediatric cardiac centers.

Aims Sudden cardiac death (SCD) risk can be managed by implantable cardioverter defibrillators (ICD). Defibrillation shocks can be delivered via ICD generator and/or intracardiac or subcutaneous coil configurations. We present our single-centre use of childhood ICDs. Methods and results Twenty-three patients had ICD implantation, with median age and weight of 12.96 years and 41.35 kg. Indications included eight long QT; four hypertrophic cardiomyopathy; three Brugada syndrome; two idiopathic ventricular fibrillation; two post-congenital heart repair; two family history of SCD with abnormal repolarization; one catecholaminergic polymorphic ventricular tachycardia; and one left ventricle non-compaction. Twelve had out of hospital cardiac arrests prior to implantation. Techniques included 13 conventional ICD implants (pre-pectoral device with endocardial leads), 7 with subcutaneous defibrillation coils (sensing via epicardial or endocardial leads tunnelled to the ICD), and 3 with exclusive subcutaneous ICD (sensing and defibrillation via the same subcutaneous lead). Satisfactory defibrillation efficacy and ventricular arrhythmia sensing was confirmed at implantation. Follow-up ranged from 0.17 to 11.08 years. One child died with the ICD in situ . Ten children received appropriate shocks; five on more than one occasion. Five received inappropriate shocks (for inappropriate recognition of sinus tachycardia or supraventricular tachycardia). Five children underwent six further interventions; all had intracardiac leads. Conclusion Innovative shock delivery systems can be used in children requiring an ICD. The insertion technique and device used need to accommodate the age and weight of the child, and concomitant need for pacing therapy. We have demonstrated effective defibrillation with shocks delivered via configurations employing subcutaneous coils in children.

We report an infant with severe long-segment tracheal stenosis in whom the posterior trachea was formed by complete cartilage rings and the anterior trachea was almost totally formed by a solid cartilage plate. The child was successfully treated initially by complete resection of the trachea and primary end-to-end repair and subsequently with tracheal homograft transplantation for secondary stenosis.

OBJECTIVES To examine the midterm clinical outcome of pulmonary valve replacement (PVR) with prosthetic valves. METHODS We reviewed 37 consecutive patients who underwent PVR with biological prosthetic valves between September 1999 and June 2010. The median age was 22.6 years (range: 6 to 70 years; three children). The primary diagnosis was Tetralogy of Fallot in 20 patients (54%). Valve pathology was regurgitation in 27 patients (72.9%). Cardiac surgery had been previously performed in 35 patients (94.5%). The median size of the prosthesis was 25 mm (range: 21 to 31 mm). The median follow-up was 42 months (range: 1.2 to 129 months). RESULTS There were no early valve-related deaths. Hospital mortality was 2.7% (n = 1) and no patient required early rereplacement of prosthesis. Two patients required permanent pacemaker insertion. During follow-up, there was no late death, reoperation for structural valve degeneration, or valve thrombosis. Only one patient required repeated operation for endocarditis at 37 months follow-up. The actuarial survival at 5 years was 95.1 ± 3.8%. Overall freedom from reoperation after PVR at 5 years was 93.0 ± 8.6%. At last follow-up, 34 patients (91.8%) were NYHA class I versus 20 patients (54%) preoperatively (p < 0.05). In the 35 surviving patients who did not undergo redo-PVR, there was no-mild regurgitation and the peak PV gradient was 20.4 ± 10.2 mm Hg (16.2 ± 9.3 mm Hg preoperatively). Thirty-two patients (91.4%) had good right ventricular function compared with 26 patients (74.2%) preoperatively. CONCLUSIONS PVR with biological prosthetic valves can be performed with good midterm survival, functional status, and haemodynamics.

To the Editor: I read with interest the paper by Manoly and colleagues1 about their coronary extension technique, whereby they used a strip of pulmonary artery anteriorly and an aortic flap posteriorly in a 19-year-old woman who had Bland-White-Garland syndrome. Although our paper was not cited, my colleague and I had described this technique in 1992. We used it in a 21-month-old girl whose anomalous left coronary artery (LCA) arose from the anterior sinus of the pulmonary trunk.2 Because the patient's aortic defect was rather small, we did not patch it. I want to report on the long-term outcome in our patient. An aortogram showed an unobstructed LCA when she was 5 years old. Catheterization data at that time showed no significant pressure gradient in the right ventricular outflow tract (right ventricular pressure, 40/6 mmHg; pulmonary artery pressure, 36/13 mmHg). The patient is now 28 years old and is doing well, without symptoms or reoperation. This method, which we now call the double-flap method, produced good early and long-term results and is a useful adjunctive procedure for 2-coronary-system reconstruction when the LCA ostium is distant from the aorta.

We describe the case of a young man aged 19 years with a double inlet left ventricle associated with transposition of the great arteries, and a mechanical aortic valve requiring anticoagulation, who presented with massive haemoptysis. At cardiac catheterisation, there were multiple feeder vessels to a bleeding leash surrounding and communicating with his left main bronchus. Despite occlusion of the larger feeder vessels, he continued to have massive haemoptysis. We describe a novel surgical strategy of denuding the peribronchial vessels through a left lateral thoracotomy. This successfully stopped his bleeding.

Several repair techniques for Ebstein's anomaly include aggressive mobilization of the anterior leaflet and the construction of a monobicuspid valve coapting against the interventricular septum. This can prove difficult when leaflet tissue is of poor quality or several fenestrations reduce leaflet surface. We present a simple and highly reproducible technique for restoring tissue continuity of the anterior leaflet that could represent a possible addendum to the existing array of surgical solutions. Two surgical cases are described. Both patients showed good valve function and trivial tricuspid regurgitation at short-term follow-up.

This is the first report of the construction of a modified Blalock-Taussig shunt using a Contegra® conduit reinforced with Goretex in a 4-year-old boy. The patient had a complex cardiac history with a primary diagnosis of pulmonary atresia, hypoplastic right ventricle (RV) and RV-coronary artery fistula.