Superior Vena Cava Inflow Following Repair for Anomalous Right Pulmonary Venous Drainage in Children.

Risk of superior vena cava (SVC) obstruction following repair of anomalous right upper pulmonary veins in children is unclear. The incidence and outcome of subclinical obstruction remained unknown. Retrospective single institutional study (07/1993-02/2017) in a pediatric population (N = 42, median age 3.9-year, range 0.1-15.3 years). 33 (79%) children had repair without SVC translocation ("non-Warden") and 9 (21%) had Warden-type surgery. Echocardiographic SVC obstruction was defined as (I) turbulent flow across SVC and (II) continuous flow pattern without return to baseline velocity (0 m/s); severe obstruction was defined as loss of distinct biphasic profile ± mean gradient ≥ 5 mmHg. 3 (7%) patients required intra-operative revision due to obstruction (non-Warden: 1, Warden: 2). After discharge, 2 (5%) patients required reintervention (3 and 6-month post-op) for severe symptomatic obstruction (non-Warden: 1, Warden-type: 1). Both patients responded to balloon angioplasty with symptomatic resolution (one required repeat catheter reintervention). 10 (24%) patients had subclinical echocardiographic obstruction (2, 22% Warden vs. 8, 24% non-Warden; p = 1.0; 8 of 10 patients had mild gradient), which resolved and remained well without reintervention. At follow-up (mean 7.2-year, range 0-23 years), all patients were alive. Freedom from SVC reintervention at 10 and 20-year is 95% (97% at 10, 20-year in non-Warden and 89% at 5, 8-year in Warden-type group; log-rank p = 0.34). Surgical repair for anomalous right upper pulmonary veins is associated with risk of SVC obstruction in children. The need for reintervention for severe obstruction is rare at late follow-up. Patients with subclinical obstruction remain asymptomatic and demonstrate echocardiographic improvement.

Extracorporeal membrane oxygenation in pediatric cardiac surgery: A retrospective review of trends and outcomes in Scotland.

INTRODUCTION: Around 3.2%-8.4% of patients receive venoarterial (VA) extracorporeal membrane oxygenation (ECMO) support after pediatric cardiac surgery. The desired outcome is "bridge-to-recovery" in most cases. There is no universally agreed protocol, and given the associated costs and complications rates, the decisions as of when and when not to institute VA ECMO are largely empirical. METHODS: A retrospective review of the ECMO database at the Scottish Pediatric Cardiac Services (SPCS) was undertaken. Inclusion criterion encompassed all children (<16 years of age) who were supported with VA ECMO following cardiac surgery between January 2011 and October 2016. The timing of ECMO support was divided into three distinct phases: "endofcase" or intheatre ECMO for patients unable to effectively wean from cardiopulmonary bypass (CPB), ECMO for cardiopulmonary resuscitation ("ECPR"), and Intensive Care Unit ECMO for "failing maximal medial therapy" following cardiac surgery. The patients were analyzed to identify survival rates, adverse prognostic indicators, and complication rates. RESULTS: We identified 66 patients who met the inclusion criterion. 30day survival rate was 45% and survival rate to hospital discharge was 44% (the difference represents one patient). On followup (median: 960 days, range: 42-2010 days), all survivors to hospital discharge were alive at review date. "End-of-case" ECMO showed a trend toward better survival of the three subcategories ("end of case," ECPR, and ECMO for "failing maximal medical therapy" survival rates were 47%, 41%, and 37.5%, respectively, P = 0.807). The poorest survival rates were in the younger children (<6 months, P = 0.502), patients who had prolonged CPB (P = 0.314) and aortic crossclamp times (P = 0.146), and longer duration of ECMO (>10 days, P = 0.177). CONCLUSIONS: Allcomers VA ECMO following pediatric cardiac surgery had survival to discharge rate of 44%. Elective "end-of-case" ECMO carries better survival rates and therefore ECMO instituted early maybe advantageous. Prolonged ECMO support has a direct correlation with mortality.

Does a balloon atrial septostomy performed before arterial switch surgery increase adverse neurological outcomes?

This best evidence topic in congenital cardiac surgery was written according to a structured protocol. The question addressed was whether the use of balloon atrial septostomy (BAS) before the arterial switch surgery for transposition of the great arteries (TGA) improved the final outcome. Altogether more than 251 papers were found using the reported search, of which 12 represented the best evidence to answer the clinical question. The search was further limited to recent articles since the results have improved compared with previous years, due to newer equipment and techniques. This narrowed the search to five papers that have focused on this issue since 2006 when a study of 29 term neonates identified BAS as major risk factor for focal brain injury and reinvigorated the debate of adverse neurological outcome especially in the context of the fact that total correction by the arterial switch procedure is routine in neonates now. Subsequently, a prospective study of 64 newborn infants followed by another study of 26 neonates with TGA, have shown no association between BAS and brain injury. Similarly, in a study of more than 2000 cases of dTGA, no association has been found between BAS and increased risk of clinical stroke either in the neonatal period or in follow-up hospitalizations. On the other hand, another nationwide data analysis of 8681 patients with TGA, has shown increased risk of stroke in patients undergoing BAS but it could only show association and not establish causation of the complication. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated.

Cystatin C: influence of perfusion and myocardial injury on early (<24 h) renal function after pediatric cardiac surgery.

BACKGROUND: Cardiopulmonary bypass (CPB)-associated renal dysfunction following cardiac surgery is well recognized. In patients with renal disease, cystatin C has emerged as a new biomarker which in contrast to creatinine (Cr) is sensitive to minor changes in glomerular filtration rate (GFR). AIM: We utilized cystatin C to investigate the association of CPB perfusion parameters with acute renal injury after pediatric cardiac surgery. METHODS: Twenty children, aged 4-58 months (AVSD, n = 7; VSD, n = 9; and ASD, n = 4), were prospectively studied. Glomerular filtration rate was quantified postoperatively by creatinine clearance (first and second 12-h periods; CrCl(0-12) and CrCl(12-24) ). Serum cystatin C and Cr were measured preoperatively and on days 0-3. Recorded CPB parameters included bypass duration (BP), perfusion pressure (PP), lowest pump flow (Q(min) ), lowest hematocrit, and corresponding lowest oxygen delivery (DO(2 min) ). Myocardial injury was determined by troponin-I. RESULTS: Postoperatively, GFR remained unchanged (CrCl(0-12) 63.6 ± 37.0 vs CrCl(12-24) 65.1 ± 27.5; P = 0.51) and only correlated with cystatin C (CrCl(0-12) vs cystatin C(Day 0) [r = 0.58, P = 0.018] and Cr(Day 0) [r = 0.09, P = 0.735]). Cr and cystatin C increased postoperatively to peak on days 2 and 3, respectively (Cr(PreOp) 31 ± 6.9 vs Cr(Day 2) 36.9 ± 12.2, P = 0.03; cystatin C(Day 0) 0.83 ± 0.27 vs cystatin C(Day 3) 1.45 ± 0.53, P = 0.02). Increased cystatin C was significantly associated with BP (P = 0.001), mean PP (P = 0.029), Q(min) (P = 0.005), troponin-I (P < 0.001), and DO(2 min) <300 ml·min(-1) ·m(-2) (P = 0.007). Receiver-operator cutoff >1.044 mg·l(-1) for cystatin C exhibited 100% sensitivity and 67% specificity for detecting renal dysfunction, defined as GFR <55 ml·min(-1) ·1.73 m(-2). CONCLUSIONS: Cystatin C is a sensitive marker of early renal dysfunction following pediatric heart surgery. Variations in bypass parameters, myocardial injury, and ultimately critical oxygen delivery are significantly associated with the degree of renal impairment.

Myocardial expression of heat shock protein 70i protects early postoperative right ventricular function in cyanotic tetralogy of Fallot.

BACKGROUND: Right ventricular dysfunction occurs after tetralogy of Fallot repair and may relate to greater myocardial vulnerability to ischemia-reperfusion injury in cyanotic patients. The inducible form of heat shock protein 70 (HSP-70i), a molecular chaperone, is upregulated in response to cellular stress and limits myocardial injury against ischemia-reperfusion. We evaluated the myocardial expression of HSP-70i and its relation to right ventricular function and clinical outcome in patients with tetralogy of Fallot undergoing corrective surgery. METHODS: Twenty patients with tetralogy of Fallot were studied: 10 cyanotic (group Cy) and 10 noncyanotic (group noCy). Western blot was used to quantify HSP-70i from resected right ventricular outflow tract myocardium at baseline and subsequent ischemic time. Biventricular function was quantified by tissue Doppler echocardiography and compared with that of 15 age-matched healthy children. Postoperative systemic perfusion was assessed by mixed venous oxygen saturation, oxygen extraction ratio, and lactate. RESULTS: Group Cy had thicker septum (median 0.85 vs 0.66 cm; P = .01) and longer crossclamp time (median 100.0 vs 67.5 minutes; P = .004). There were no difference in HSP-70i between groups at baseline (4.12 vs 3.44 relative optical density; P = .45) or subsequent ischemic time. Preoperative biventricular systolic function was reduced in patients with tetralogy compared with controls with further postoperative right ventricular impairment. Group Cy had higher troponin-I levels (median 16.5 vs 11.1 ng/mL; P = .04) and inotrope scores (14.0 vs 6.5; P = .05) but no differences in ventricular function, mixed venous oxygen saturation, oxygen extraction ratio, and lactate between groups. In group Cy, baseline HSP-70i correlated with better postoperative right ventricular function (rho = 0.80; P = .009), mixed venous oxygen saturation (rho = 0.68; P = .04), and oxygen extraction ratio (rho = -0.71; P = .03). These relationships were absent in group noCy. CONCLUSIONS: The association of HSP-70i expression with improved right ventricular function and systemic perfusion suggests an important cardioprotective effect of HSP-70i in cyanotic tetralogy of Fallot.

Synergistic interaction between right ventricular mechanical dyssynchrony and pulmonary regurgitation determines early outcome following tetralogy of Fallot repair.

OBJECTIVE: The ability of the right ventricle to tolerate acute pulmonary regurgitation (PR) following tetralogy of Fallot (TOF) repair is variable and the mechanisms that underlie this are not completely understood. We hypothesise that dyssynchronous wall mechanics affects the RV tolerance to postoperative PR with adverse effect on early surgical outcome. METHODS: Twenty-four TOFs (mean age 19.5+/-15.5 months) undergoing elective repair were prospectively recruited. Ventricular wall mechanics was studied by tissue Doppler echocardiography following induction (preop) and postoperative day one (POD1) and compared with a control group (10 VSD/AVSD). Segmental dyssynchrony, defined as out-of-phase peak myocardial contraction, was determined at the base, mid, apical segments of the septum, RV and LV free walls and scored by the total number of affected segments. PR was graded from absent to severe and RV dimension was quantified by end-diastolic area index (RVEDAI). Cardiac index (CI) was measured by pulse contour cardiac output analysis. Outcome measures were CI, mixed venous oxygen saturation (SvO2), lactate, and duration of ventilation and critical care stay. RESULTS: Preoperatively, biventricular free-wall motion was synchronous in both groups. Following surgery, TOF developed RV-septal dyssynchrony (>2 segments in 11 (46%) vs none in control, p=0.01), while the LV free wall remained normal in both groups. RV-septal dyssynchrony correlated with the ventilation time (rho=0.69, p=0.003), critical care stay (rho=0.58, p=0.02) in the presence of PR (n=16), but not with other outcome measures. The relationships between dyssynchrony and early outcome were not seen when PR was absent. In the presence of PR, median RVEDAI was greater with higher dyssynchrony score (>3 segments; p=0.009). The degree of PR did not affect critical care/ventilation time or RVEDAI. The presence of transannular patch (p=0.007) or at least moderate PR (p=0.01) was associated with a more severe dyssynchrony. CONCLUSIONS: Dyssynchronous RV-septal wall mechanics occurs early after Fallot repair. The magnitude of dyssynchrony appears to interact synergistically with pulmonary regurgitation to influence RV dimension and early outcome.

Tissue Doppler imaging following paediatric cardiac surgery: early patterns of change and relationship to outcome.

In this study, tissue Doppler imaging (TDI) was used to assess changes in ventricular function following repair of congenital heart defects. The relationship between TDI indices, myocardial injury and clinical outcome was explored. Forty-five children were studied; 35 with cardiac lesions and 10 controls. TDI was performed preoperatively, on admission to paediatric intensive care unit (PICU) and day 1. Regional myocardial Doppler signals were acquired from the right ventricle (RV), left ventricle (LV) and septum. TDI indices included: peak systolic velocities, isovolumetric velocities (IVV) and isovolumetric acceleration (IVA). Preoperatively, bi-ventricular TDI velocities in the study group were reduced compared with normal controls. Postoperatively, RV velocities were significantly reduced and this persisted to day-1 (PreOp vs. PICU and day-1: 7.7+/-2.2 vs. 3.4+/-1.0, P<0.0001 and 3.55+/-1.29, P<0.0001). LV velocities initially declined but recovered towards baseline by day-1 (PreOp vs. PICU: 5.31+/-1.50 vs. 3.51+/-1.23, P<0.0001). Isovolumetric parameters in all regions were reduced throughout the postoperative period. Troponin-I release correlated with longer X-clamp times (r=0.82, P<0.0001) and reduced RV velocities (r=0.42, P=0.028). Reduced pre- and postoperative LV velocities correlated with longer ventilation (PreOp: r=0.54, P=0.002; PostOp: r=0.42, P=0.026). This study identified reduced postoperative RV velocities correlated with myocardial injury while reduced LV TDI correlated with longer postoperative ventilation.

Effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension and congenital cardiac defects.

OBJECTIVE: Sildenafil (Viagra, Pfizer) is being increasingly used to treat pulmonary hypertension in children. However, there are limited data available to suggest dosage regimens. The purpose of this study was to determine the effects of escalating doses of sildenafil on hemodynamics and gas exchange in children with pulmonary hypertension because of congenital cardiac defects. DESIGN: Prospective, observational study. SETTING: Pediatric intensive care unit in a tertiary care children's hospital. PARTICIPANTS: Ten children with pulmonary hypertension because of congenital cardiac defects who were in the intensive care unit and on nitric oxide after cardiac surgery. INTERVENTION: Patients received sildenafil every 4 hours via a gastric tube in incremental doses of 0.5 mg/kg, 1 mg/kg, 1.5 mg/kg, and 2.0 mg/kg along with nitric oxide during their stay in the intensive care unit until they were extubated. Hemodynamic and arterial blood gas measurements were taken before (baseline) and 60 minutes after the administration of sildenafil. MEASUREMENTS AND MAIN RESULTS: All doses of sildenafil caused significant reduction in pulmonary artery pressure with no significant effect on systemic arterial and central venous pressures. Arterial partial pressure of oxygen was decreased after a 2.0 mg/kg dose of sildenafil but not significantly. No significant differences were found among the 4 doses. CONCLUSION: For the treatment of pulmonary hypertension in children with congenital cardiac defects, a 0.5 mg/kg dose of sildenafil every 4 hours is therapeutically as effective as a 2.0 mg/kg dose every 4 hours. However, a large dose-ranging and pharmacokinetic study of sildenafil in children with pulmonary hypertension because of congenital cardiac defects is needed to validate the safety and efficacy of the dose-range and dosing interval suggested by this study.

Impact of stentless aortic valves on left ventricular function and hypertrophy: current best available evidence.

Past four decades have seen a gradual evolution in aortic valve replacement surgery. The ideal valve substitute should combine central flow, low transvalvular gradient, low thrombogenicity, durability, easy availability, resistance to infection, freedom from anticoagulation, and easy implantability. Although there are several types of valves available to replace the diseased aortic valve-autograft, allograft, xenograft, mechanical, and bioprosthetic valves-none is ideal. On one end of the spectrum is the pulmonary autograft, which comes closest to achieving these goals, but creates a double valve procedure for single valve disease, while on the other end are the mechanical valves and stented tissue valves, which allow easy "off the shelf" availability as well as easy implantability but are limited by the potential drawback of causing intrinsic obstruction to some extent because of the space occupied by the stent and sewing ring. Stentless xenograft aortic valves have been developed as a compromise between these ends of the valve spectrum. Stentless aortic valves have been reported to provide more physiologic hemodynamic behavior and cause more timely and thorough regression of ventricular hypertrophy. This review article attempts to evaluate current best available evidence from randomized controlled trials to assess the impact of stentless aortic valves on left ventricular function and hypertrophy.

Is sildenafil effective for treating pulmonary hypertension after pediatric heart surgery?

A best evidence topic in pediatric cardiac surgery was written according to a structured protocol. The question addressed was whether sildenafil was effective in treating postoperative pulmonary hypertension after pediatric heart surgery. Altogether 28 papers were found using the reported search, of which three represented the best evidence to answer the clinical question. The author, journal, date and country of publication, patient group studied, study type, relevant outcomes, results, and study weaknesses were tabulated. We conclude that oral sildenafil may be useful in reducing pulmonary vascular resistance and can be considered for treatment of postoperative pulmonary hypertension after pediatric heart surgery. However, evidence from a large, multicenter, randomized controlled trial is needed to validate the safety and efficacy of sildenafil for use in postoperative pediatric cardiac surgical patients.

Current status of the Contegra conduit for pediatric right ventricular outflow tract reconstruction.

Reconstruction of the right ventricular outflow tract (RVOT) is needed in a wide variety of congenital heart diseases at the time of primary repair, or later for replacement of existing valves or conduits. Ideally, the conduit or valve needed for such reconstruction should be formed of autologous tissue that grows, resists infection, lasts for the life span of the patient, and is readily available in all sizes. Such a conduit is not available, though several alternatives have been used, none of which is without potential drawbacks. One alternative--the Contegra bovine jugular vein conduit (Medtronic, Inc., Minneapolis, MN, USA)--was introduced in 1999 and has gained widespread application, with increasing enthusiasm for its use. The Contegra conduit consists of a bovine jugular vein with an incorporated trileaflet valve. The conduit tissue is extremely pliable and offers optimal conditions for surgical handling. Moreover, the proximal tubular segment allows construction of the proximal anastomosis to the right ventricle, without the use of additional material. Increasingly, experience with the Contegra conduit is being published; hence, a literature search was conducted to evaluate available evidence on current use of the device in pediatric RVOT reconstruction.

Anomalous left coronary artery, ventricular septal defect, and double aortic arch.

Anomalous origin of the left coronary artery from the pulmonary artery generally occurs in isolation. We report an infant diagnosed with double aortic arch and ventricular septal defect who was found to have an anomalous origin of the left coronary artery from the pulmonary artery at surgery. Successful ventricular septal defect repair, division of the arch, and aortic reimplantation of the left coronary artery was performed. This article describes a combination of lesions previously not described, highlights the altered presentation when multiple lesions coexist, discusses the propensity to miss the anomalous coronary, and reviews the literature that demonstrates the consequences of overlooking this defect.

Pediatric mitral valve replacement: incremental risk factors impacting survival and reintervention.

BACKGROUND AND AIM OF THE STUDY: Predictors of survival and reintervention in children undergoing mitral valve replacement (MVR) were assessed. METHODS: A total of 37 children underwent MVR, with a mean prosthesis size of 22.5 mm. RESULTS: Six patients died during the study. Actuarial and event-free survivals (including operative mortality) at 10 and 20 years were 85 and 85%, and 55 and 37%, respectively. The linearized rate of bleeding was 0.67% per patient-year (pt-yr). There were no instances of thromboembolism, endocarditis or structural valve dysfunction. Six patients required reintervention for somatic outgrowth at a mean postoperative interval of 104 months. Freedom from reintervention at five, 10 and 20 years was 92, 73 and 48%, respectively. There were no deaths or complications following reintervention. The mean follow up was 96 months (range: 3 to 276 months); total cumulative follow up was 296 pt-yr. Multivariate predictors of mortality were age < 6 months, NYHA class IV, congestive cardiac failure, internal orifice diameter (IOD) < 14 mm and effective orifice area (EOA) < 1.5 cm2. Predictors for reintervention were age < 6 months (p = 0.0021), predicted annulus size < 16 mm (p < 0.0001), IOD < 18 mm (p = 0.05), IOD index (IODI) > 50 mm/m2 (p = 0.007), EOA < 2.5 cm2 (p = 0.05), EOA index (EOAI) > 7 cm2/m2 (p < 0.0001) and prosthesis size-body surface area (BSA) index (SI) > 69 mm/m2 (p = 0.007). CONCLUSION: Early mortality occurs in neonates and infants with associated lesions, particularly atrioventricular septal defect. Risk factors for mortality include age, NYHA class, and small IOD and EOA. Risk factors for reintervention include age < 6 months, small predicted annulus size, IOD and EOA, IODI > 50 mm/m2, EOAI > 7 cm2/m2 and SI > 69 mm/m2.

Mechanical aortic valve replacement: long-term outcomes in children.

BACKGROUND AND AIM OF THE STUDY: Early and late outcomes following mechanical aortic valve replacement (AVR) in children were analyzed. METHODS: Between January 1980 and December 2003, 55 patients underwent mechanical AVR at the authors' institution. Of these patients, 12 had aortic regurgitation (AR), 31 had aortic stenosis (AS), and 12 had mixed disease. Preoperatively, 25 patients (45.5%) were in NYHA classes III and IV. Among patients, 22 had a previous valvotomy and 19 had associated cardiac defects. Isolated AVR was performed in 37 cases. Twelve patients required root enlargement procedures, two had aortic root replacement, three had a double valve replacement, and one patient had a concurrent mitral annuloplasty. The mean prosthesis size was 22.6 mm (range: 16-31 mm). Mean follow up was 12.28 +/- 6 years (range: 1-23 years); total follow up was 665 patient-years (pt-yr). RESULTS: There was one late death. Actuarial survival at 20 years was 98 +/- 2%. Event-free survival at one, five and 20 years was 96 +/- 3%, 92 +/- 4% and 88 +/- 5%, respectively. Four patients required reoperation (two for valve outgrowth, one each for paravalvular leak and prosthetic valve endocarditis (PVE)). Freedom from reintervention at one, five and 20 years was 98 +/- 2%, 96 +/- 3% and 92 +/- 4%, respectively. There was one event of anticoagulation-related hemorrhage. Freedom from anticoagulant-related hemorrhage at 20 years was 98 +/- 2%, and freedom from PVE at five and 20 years was 98 +/- 2% and 96 +/- 3%, respectively. There were no instances of thromboembolism or structural valve dysfunction. Linearized rates of bleeding and endocarditis were 0.15 and 0.3% per pt-yr, respectively. At follow up, 54 children were in NYHA classes I or II. CONCLUSION: Mechanical AVR, with aortic root enlargement if necessary, is associated with low mortality and morbidity, and is an excellent treatment option in children. Late embolic and hemorrhagic complications are infrequent in the current era.

Surgical repair of double aortic arch: 16-year experience.

Double aortic arch (DAA) is a complete form of vascular ring causing tracheoesophageal compression. We analyzed long-term results of a series of DAAs, over a period of 16 years. Between 1987 and 2003, 29 children underwent surgery for airway and/or esophageal compression secondary to a DAA. Dominant symptoms were stridor, dysphagia, choking episodes, and life-threatening apneic spells (n = 7). Diagnosis was established by barium studies, bronchoscopy, echocardiogram, angiogram, computed tomography (CT), and magnetic resonance imaging (MRI). Seven patients had concurrent cardiac anomalies. Two children had an associated tracheoesophageal fistula. Surgery was accomplished by left thoracotomy (n = 25), right thoracotomy (n = 2) or median sternotomy (n = 2). The operative mortality was zero. There was one late death due to respiratory failure. Four (13.8%) patients had a surgical complication (chylothorax, 3 cases; acute renal failure, 1 case). Follow-up (mean 7.1 years; range 6 months to 16 years) was complete in all patients, and showed complete improvement in 22 patients and partial improvement in 6 patients. Early surgical repair of DAA is associated with low mortality, and results in marked symptomatic relief in most patients. Patients with tracheomalacia or associated asthma, constitute a high-risk group and may manifest persistent symptoms and require adjunctive procedures.

Congenital lung malformations--antenatal and postnatal evaluation and management.

We reviewed our institutional experience with pulmonary resection for congenital bronchopulmonary malformations and analysed the management and outcome of pregnancies with a prenatal diagnosis of congenital lung malformations. Between January 1993 and December 2003, 31 patients underwent evaluation and pulmonary resection for bronchopulmonary malformations. Common clinical presentations were respiratory distress (9), respiratory infections/pneumonias (22), and dyspnoea (9). Diagnostic modalities included chest radiography, CT scan (22), MRI scan (7), arteriography (1), and bronchoscopy (5). There were 13 congenital cystic adenomatoid malformations (CCAM), six pulmonary sequestrations, three bronchogenic cysts, and nine congenital lobar emphysemas (CLE). Fifteen patients who underwent resection were diagnosed by antenatal ultrasound. No foetus had hydrops or associated malformations. No pregnancy was terminated. There was no foetal demise. Regression of the sonographic appearance was observed in six cases. Amniotic puncture was required for hydramnios in three cases. Eight emergency resections were performed (CCAM 4; CLE 3; Bronchogenic cyst 1). Surgical procedures included 24 lobectomies, one right middle lobectomy with a wedge resection of the right lower lobe, one completion right lower lobectomy, four sequestrectomies, one mediastinal mass excision and one wedge resection for a bronchogenic cyst. There were no deaths. Postoperative complications included: persistent air leak (n=2; one requiring completion lobectomy) and pneumothorax (1). Persistent mild symptoms were present in five patients, at long-term follow-up. Congenital cystic adenomatoid malformation and congenital lobar emphysema were the commonest congenital anomalies. Congenital lung malformations are increasingly diagnosed antenatally, sometimes necessitating emergent surgical resection. The natural history is variable. All infants with a prenatal diagnosis require postnatal evaluation. Patients should be evaluated for associated disorders. The presence of mass effects is an indication for therapeutic decompression. The risk of pulmonary compression, infection and malignant degeneration makes resection imperative, even in asymptomatic patients. Lobectomy is the procedure of choice, is well tolerated, and leads to excellent outcomes.

Ascending aortic origin of a branch pulmonary artery--surgical management and long-term outcome.

OBJECTIVE: Ascending aortic origin of a branch pulmonary artery (AOPA, hemitruncus arteriosus) is a rare congenital malformation. While there have been isolated case reports, larger series, relating to long-term outcomes following surgery are few. This article analyses the surgical results of a series of nine patients, over a period of 29 years. METHODS: Between 1974 and 2003, nine patients [neonates, 6; infants, 3; male, 5; female, 4] were operated on for AOPA. Median age at presentation was 14 days (range birth to 231 days). Six [corrected] patients (group 1) had associated simple lesions like patent ductus arteriosus or right aortic arch. Three patients (group 2) had complex lesions with right ventricular outflow tract obstruction. One patient (group 2) had DiGeorge syndrome. All patients except group 2 presented with congestive cardiac failure and, in addition one had pre-operative coronary ischemia. Diagnosis was established by angiocardiography in two patients and by echocardiography in seven [corrected] The median age at operation was 28 days (range 7-365). Follow-up period ranged from 7 months to 20.5 years (median 9 years). RESULTS: All nine patients had an anomalous right pulmonary artery (RPA) arising from the proximal ascending aorta, while the left branch was of right ventricular origin. All had evidence of pulmonary hypertension or elevated right ventricular pressure pre-operatively. There was no operative mortality. Of eight patients who had direct anastomosis of the RPA to the main pulmonary artery, one required patch enlargement and another required stenting of an anastomotic stenosis. One patient had a RV-RPA conduit, which required replacement 8, 13, and 14 years later. At follow-up, all patients were alive. All patients in group 1 had normal haemodynamic function and were in NYHA class I. In group 2, all were in NYHA class II with evidence of right ventricular hypertrophy. Four patients had post-operative ventilation-perfusion scans which showed satisfactory perfusion to both lungs. CONCLUSIONS: Early surgery is indicated in this lesion and is compatible with good long-term outlook. Surgical repair should not be deferred for corrective procedures of associated cardiac anomalies.

Double outlet left ventricle, transposition of great vessels and pulmonary stenosis.

A 4 year-old boy with double outlet left ventricle, transposition of the great vessels, severe pulmonary and subpulmonary stenosis, and subaortic ventricular septal defect underwent successful surgical intervention. The surgical techniques and management plan for double-outlet left ventricle are reviewed.

Duroflo II heparin bonding does not attenuate cytokine release or improve pulmonary function.

BACKGROUND: Comparison of the cytokine generation and leukocyte activation properties of Duroflo II heparin bonded bypass circuit (Baxter Healthcare Corp, Compton, UK) and the conventional cardiopulmonary bypass circuit. Attempt to correlate these to pulmonary dysfunction postoperatively. METHODS: Forty patients undergoing elective, isolated coronary artery bypass grafting were randomly allocated to have either plain extracorporeal circuits (group C) or heparin bonded extracorporeal circuits (group H). Full systemic heparinization was used in all patients. The inflammatory response was assessed by measuring plasma levels of interleukin-6, interleukin-8, interleukin-10, and polymorphonuclear elastase. Gas exchange was assessed by measuring the PaO2/FIO2 ratio. RESULTS: Significant impairment of oxygenation was seen in both groups with the lowest values at the end of the operation before a gradual return to normal during the next 6 hours. There were no differences between the groups in gas exchange or times to extubation. There were significant elevations in all the cytokines, with interleukin-6 levels peaking at 4 hours in group H and 24 hours in group C, before starting to return to normal at 48 hours. The patterns of interleukin-8 and interleukin-10 rise were identical in the two groups. Polymorphonuclear elastase reached a peak at the end of the operation in group H and remained elevated up to 24 hours, whereas levels continued to rise in group C up to 4 hours. There were no significant differences in levels between groups at any time. There were no differences between the groups in blood loss or blood product usage. CONCLUSIONS: Cardiopulmonary bypass induces a systemic inflammatory response with release of cytokines and activation of leukocytes. This correlates with the severe deterioration in pulmonary gas exchange from preoperative levels up to 6 hours postoperatively (p < 0.05). In the presence of systemic heparinization, Duroflo II heparin bondingtf the circuits has minor effects on the pattern of evolution of this inflammatory response.