Clinical and angiographic follow-up of coronary artery fistula interventions in children: techniques and classification revisited.

BACKGROUND: Transcatheter closure of coronary artery fistula has emerged as a safe and effective alternative to surgery. However, follow-up angiographic data after closure of the coronary artery fistula is extremely limited. We report our clinical and angiographic follow-up of children who underwent either transcatheter or surgical closure. METHOD: Clinical profile, echocardiography parameters, and closure technique were retrospectively reviewed from the hospital charts. Since 2007, 15 children have been intervened and followed up with electrocardiography, echocardiography, and angiography. RESULTS: A total of 15 children (six girls), with mean age of 6.7±5.4 years and weighing 16.3±9.8 kg, underwent successful closure (transcatheter=13, surgical=2) without periprocedural complication. Coronary artery fistula arose from the right (n=7) and left coronary artery (n=8) and drained into the right atrium or the right ventricle. Transcatheter closure was carried out using a duct occluder. Of the patients, two underwent surgical closure of the fistula on a beating heart. At 31.8±18.7 months follow-up, all the children were asymptomatic and had no evidence of myocardial ischaemia or infarction. However, follow-up angiography revealed thrombotic occlusion of fistula with the patent parent coronary artery in those having branch coronary artery fistula, and five of seven patients with parent coronary artery fistula had near-complete occlusion of fistula extending into the native coronary artery. CONCLUSION: Follow-up angiography revealed a high incidence of parent artery occlusion when the fistula was arising from the native artery and not from one of its branches. Coronary artery fistula intervention of the parent coronary artery fistula always carries the potential risk of ischaemia, unless the distal-most exiting segment is the primary site of occlusion.

A rare case report of corrected transposition of the great arteries in association with tuberous sclerosis and cardiac rhabdomyomas.

The neuro-cutaneous syndrome tuberous sclerosis is commonly associated with rhabdomyomas in various organs including the heart. We are reporting a rare case of a 7-month old male child with congenitally corrected transposition of the great arteries associated with tuberous sclerosis and cardiac rhabdomyomas. To our knowledge, this rare association has not been reported so far.

Aberrant subclavian artery origin in tetralogy of Fallot with pulmonary stenosis is associated with chromosomal or genetic abnormality.

We determined the relationship between aortic arch anatomy in tetralogy of Fallot with pulmonary stenosis and chromosomal or genetic abnormality, by performing analysis of 257 consecutive patients undergoing surgical repair from January, 2003 to March, 2011. Chromosomal or genetic abnormality was identified in 49 of the 257 (19%) patients. These included trisomy 21 (n = 14); chromosome 22q11.2 deletion (n = 16); other chromosomal abnormalities (n = 9); CHARGE (n = 2); Pierre Robin (n = 2); and Kabuki, Alagille, Holt-Oram, Kaufman McKusick, Goldenhar, and PHACE (n = 1 each). Aortic anatomy was classified as left arch with normal branching, right arch with mirror image branching, left arch with aberrant right subclavian artery, or right arch with aberrant left subclavian artery. Associated syndromes occurred in 33 of 203 (16%) patients with left arch and normal branching (odds ratio 1); three of 36 (8%) patients with right arch and mirror image branching (odds ratio 0.4, 95% confidence interval 0.1-1.6); seven of eight (88%) patients with left arch and aberrant right subclavian artery (odds ratio 36, 95% confidence interval 4-302); and six of 10 (60%) patients with right arch and aberrant left subclavian artery (odds ratio 8, 95% confidence interval 2-26). Syndromes were present in 13 of 18 (72%) patients with either right or left aberrant subclavian artery (odds ratio 15, 95% confidence interval 4-45). Syndromes in patients with an aberrant subclavian artery included trisomy 21 (n = 4); chromosome 22q11.2 deletion (n = 5); and Holt-Oram, PHACE, CHARGE, and chromosome 18p deletion (n = 1 each). Aberrant right or left subclavian artery in tetralogy of Fallot with pulmonary stenosis is associated with an increased incidence of chromosomal or genetic abnormality, whereas right aortic arch with mirror image branching is not. The assessment of aortic arch anatomy at prenatal diagnosis can assist counselling.

Stenting across head and neck vessels using covered stents for persisting aortic arch obstruction.

OBJECTIVE: To describe endovascular stent placement using partially covered stents to preserve flow in head and neck vessels. BACKGROUND: Endovascular stent placement has become established as a first-line therapy for native coarctation of the aorta or re-coarctation in older children and adults. Increasingly covered stents are becoming the preferred option over bare-metal stents because of the perceived lower risk of aneurysm formation. Open-cell bare-metal stents are chosen when there is a high likelihood of jailing a head and neck vessel. Here we describe partial uncovering of a covered stent before implantation to allow flow through the uncovered portion of the stent to the branch vessel but preserve the covering over the majority of the remaining stent. METHODS: We describe two cases with aortic arch hypoplasia and re-coarctation, both of which required two partially uncovered stents for a satisfactory result. CONCLUSIONS: Endovascular stent placement is becoming the preferred option in the management of coarctation of the aorta in older children and adults. Strategies to deal with transverse arch hypoplasia and multiple levels of aortic arch obstruction frequently involving branch vessels or aneurysms need to be considered before these procedures are embarked upon. Partially uncovering stents may afford more protection than using bare-metal stents in the transverse and distal arch while preserving flow in head and neck branches, and is a technically straightforward procedure.

Cardiac magnetic resonance imaging predicts cardiac catheter findings for great artery stenosis in children with congenital cardiac disease.

OBJECTIVE: To assess the cardiac catheterisation findings of all children in whom cardiac magnetic resonance imaging found great artery stenosis. METHODS: We conducted a retrospective analysis of all 45 consecutive children with congenital cardiac disease who were undergoing cardiac catheterisation for intervention on cardiac magnetic resonance-defined great vessel stenosis, between January, 2006 and August, 2008. RESULTS: Following cardiac magnetic resonance, 60 significant great vessel stenoses were identified and referred to cardiac catheterisation for intervention. All patients were catheterised within a median and interquartile range of 84 and 4-149 days, respectively, of cardiac magnetic resonance. At cardiac catheterisation, the children were aged 11.5 years - with an interquartile range of 3.8-16.9 years - and weighed 34 kilograms - with an interquartile range of 15-56 kilograms. Comparing cardiac magnetic resonance and cardiac catheterisation findings, 53 (88%) findings were concordant and seven were discordant. In six of seven (86%) discordant observations, cardiac magnetic resonance defined moderate-severe great vessel stenosis - involving three branch pulmonary arteries and three aortas. This was not confirmed by cardiac catheterisation, which revealed mild stenoses and haemodynamic gradients insufficient for intervention. In one patient, a mild, proximal right pulmonary artery narrowing was found at cardiac catheterisation, which was not mentioned in the cardiac magnetic resonance report. There was no difference between discordant and concordant groups on the basis of patient age, weight, interval between cardiac magnetic resonance and cardiac catheterisation, or type of lesion. CONCLUSION: Invasive assessment confirmed cardiac magnetic resonance-diagnosed great vessel stenosis in the majority of this cohort. The predominant discordant finding was lower catherisation gradient than predicted by morphologic and functional cardiac magnetic resonance assessment. Flow volume diversion - for example, unilateral pulmonary artery stenosis - and anaesthetic effects may account for some differences. Prospective refinement of cardiac magnetic resonance and interventional data may further improve the validity of non-invasive imaging thresholds for intervention.

A rare cause of ischemic heart failure in a neonate.

Herein, we present a case of ischemic heart failure that occurred immediately after birth in a neonate due to coronary artery fistula (CAF) from the left main coronary artery to the left atrial appendage associated with high pulmonary artery pressure. Ischemic heart failure in a neonate with a structurally normal heart is rare. Furthermore, CAF resulting in ischemic heart failure is very rare in neonates. We believe that the small CAF caused symptoms during the first few days of life due to moderate pulmonary hypertension which resulted in a low cardiac output. The coronary perfusion improved after the normalization of the pulmonary blood pressure and improvement of the cardiac output. Echocardiography is helpful when a CAF is suspected and can be confirmed using a cardiac computed tomography scan. Small CAFs are unlikely to cause symptoms in infants, provided there are no other factors affecting the cardiac output status.

Ebstein's Anomaly: "The One and a Half Ventricle Heart".

OBJECTIVE: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). METHODS: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. RESULTS: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho -0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). CONCLUSION: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

Ductus arteriosus stenting: A promising percutaneous palliation in patients with duct-dependent pulmonary circulation.

OBJECTIVES: We aimed to study the feasibility and outcomes of ductal stenting in patients with duct-dependent pulmonary blood flow (PBF). METHODS: Duct-dependent hypoxic patients with confluent pulmonary artery (PA) branches were enrolled for ductal stenting and followed regularly. RESULTS: Sixty patients, with a median age of 12 (1-1095) days and weight of 2.8 (2.2-8.9) kg, were enrolled. Median right PA (RPA) and left PA (LPA) Z-scores were -1.23 (-10.54 to 2.81) and -0.96 (-8.03 to 3.0), respectively. Mean narrowest ductal diameter was 1.73±0.57 mm and length was 12.78±3.32 mm. Sixty-four stents with mean diameter of 4.21±0.32 mm and length of 14.34±3.44 mm were deployed in 59 patients. The procedure was unsuccessful in one. Post-stenting mean oxygen saturation (SO2) increased significantly from baseline of 68.88±7.47% to 90.43±6.04% (p<0001). Complications included pulmonary edema in one patient and acute stent occlusion in another. At a median follow-up of eight (2-14) months, mean SO2 (80.04±7.54%) was significantly higher than baseline (p<0.0001). Median RPA and LPA Z-scores, 0.56 (-2.89 to 3.29) and -0.02 (-2.81 to 3.86), respectively, were significantly higher than baseline. Six patients required re-interventions (shunt in three and angioplasty in three). Six patients died, three due to sepsis and another three with worsened cyanosis due to impaired PBF, probably due to ductal occlusion. CONCLUSION: Ductal stenting is an effective palliation in patients with duct-dependent PBF. It maintains adequate SO2 and promotes balanced PA growth at mid-term follow-up.

Total anomalous systemic and pulmonary venous connection.

Total anomalous systemic and pulmonary venous connection is an extremely rare congenital cardiac anomaly. We present our unique experience of managing this complex partially diagnosed cardiac anomaly in a 16-month-old boy. The systemic venous anomaly was not detected during the initial preoperative evaluation. He was doing well on follow-up, with normal pulmonary artery pressure.

Transcatheter closure of left pulmonary artery to left atrium fistula in a neonate using amplatzer duct occluder I.

A direct-fistula type communication between branch pulmonary artery (PA) to left atrium (LA), particularly left PA to LA, is a very rare congenital cardiopulmonary disorder. Although surgical repair is the conventional treatment, transcatheter device or coil closure is feasible in selected cases of a relatively frequent variant - right PA to LA fistula. Ours is the first case of successful transcatheter closure of a large left PA to LA fistula with Amplatzer duct occluder in a cyanotic neonate by transseptal approach.

A simple surgical technique for closure of apical muscular ventricular septal defect.

OBJECTIVE: Ventricular septal defect (VSD) is among the most common congenital heart diseases encountered in pediatric cardiac patients. Apical muscular VSD constitutes nearly 2% of defects, which may or may not be associated with other congenital heart defects. The purpose of our study is to present our innovative and simple surgical technique using custom-made low-profile polytetrafluoroethylene (PTFE) single disc device for closing multiple apical muscular and isolated apical muscular VSD. METHOD: Between January 2010 and July 2013, 17 patients with isolated or multiple apical muscular VSDs with or without associated heart diseases underwent operation at our institute. The apical VSD was closed using our custom-made low-profile single disc polytetrafluoroethylene device. The operative technique and the technique used to prepare the single disc device are detailed. RESULTS: Seventeen patients of ages ranging from 3 months to 7 years underwent operation over 3 years. One 8-month-old patient with transposition of the great arteries with multiple VSDs died after 35 days due to severe pulmonary artery hypertension and sepsis. Another newborn infant with infracardiac total anomalous pulmonary venous connection with a 4-mm apical VSD also died after 3 days because this VSD could not be identified. All other patients are doing well on follow-up. CONCLUSIONS: The technique described by us has the advantage of apical VSD closure through the left ventricle without left ventriculotomy. Our technique is simple and cost-effective.

Neither age at repair nor previous palliation affects outcome in tetralogy of Fallot repair.

OBJECTIVES: The study aimed to evaluate the results following complete repair of tetralogy of Fallot (TOF) in relation to age at surgery and to assess the role of palliation in the current era. METHODS: A retrospective review of 251 consecutive patients with TOF repaired between 2003 and 2011 at the Great Ormond Street Hospital was performed. Children were divided into two groups: Group A, younger than 6 months (n = 78) and B, older than 6 months (n = 173). Early clinical outcomes and reoperation/reintervention rates were studied as well as indication for a palliation. RESULTS: There was 1 (0.4%) early and 1 (0.4%) late death after a median follow-up time of 4.5 years. Forty-three patients (17%) underwent repair after initial palliation with inter-stage mortality of 5%. Groups A and B were similar in terms of surgical approach, postoperative complications and length of stay. Significant differences were found in terms of more frequent use of a transannular patch (P = 0.05), longer surgeries (P = 0.02) and a greater proportion of palliated patients (P = 0.002) in older patients. There was no difference in rates of reoperation/reintervention between groups and following both primary and staged repair. Palliated patients were more symptomatic (duct-dependent pulmonary blood flow; P < 0.01, cyanotic spells; P < 0.01), had more extracardiac/genetic anomalies (P < 0.01), coronary anomalies (P = 0.015) and significantly smaller pulmonary annulus, right pulmonary artery (RPA) and left pulmonary artery (LPA) Z-scores (P < 0.01 for all). CONCLUSION: Age at complete repair was not linked to early clinical outcome or reoperation/reintervention rate. Palliative procedures postponed the timing of complete repair, but did not increase the reintervention rate.

Ebstein's anomaly: "The one and a half ventricle heart"

Abstract Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). Methods: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. Results: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). Conclusion: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

Midterm follow-up of arterial switch operation for transposition of the great arteries with intact ventricular septum and left-ventricular outflow tract obstruction.

OBJECTIVE: We report the mid-term follow-up of patients, who underwent arterial switch operation (ASO) for transposition of the great arteries (TGA) with intact ventricular septum and left-ventricular outflow tract obstruction (LVOTO) over a 10-year period from 2000 to 2009. METHODS: Thirteen TGA patients (3.9% of our ASO cohort) with intact ventricular septum and LVOTO underwent ASO. LVOTO was defined as pulmonary valve z-score ≤ -2.0 (n=3) or peak LVOT gradient ≥40 mmHg with (n=7) or without (n=3) anatomic subvalvar stenosis on echocardiography. Median age and weight were 14 days (range, 7-130 days) and 3.2 kg (range, 2.1-4.6 kg). The LVOT abnormalities included fibromuscular narrowing (n=5) and atrioventricular valve-related findings (n=5). LVOT clearance was achieved by resection of accessory mitral tissue (n=2) only. RESULTS: Follow-up was 100% complete. There were no early or late deaths. Freedom from re-operation for neo-aortic valve regurgitation and/or LVOTO was 100% at a median follow-up of 38 months (range, 6-115 months). All patients had functional status appropriate for their age. Three patients had mild aortic regurgitation. The median Doppler estimated LVOT systolic gradient was 12 mmHg (range, 0-18 mmHg) for the entire cohort at the latest follow-up. CONCLUSIONS: Mid-term outcomes of ASO for a highly selected group of patients with pulmonary valve annulus z-score ≤ -2.0 ≥ -0.4, resectable organic LVOTO, and dynamic peak LVOT gradient ≥40 mmHg remain satisfactory, with a need for long-term follow-up.

Outcomes and re-interventions after one-stage repair of transposition of great arteries and aortic arch obstruction.

OBJECTIVES: One-stage repair of transposition of great arteries (TGA) and aortic arch obstruction (AAO) is currently advocated, but carries formidable surgical challenges. This report presents our experience and re-interventions for residual lesions over the last 10 years. METHODS: Twenty-two patients (19.5 ± 42.4 days; range 2-206; median 10 days, 3.5 ± 0.6 kg) diagnosed with TGA (nine patients) or double outlet right ventricle (DORV) (13 patients) and AAO underwent one-stage repair. Of the nine TGA patients (two with intact ventricular septum), AAO were: two patients hypoplastic arch, one patient discrete coarctation, four patients hypoplastic arch with coarctation and two patients interrupted aortic arch. The 13 DORV patients were all of Taussig-Bing type and one showed multiple ventricular septal defects (VSDs). The degree of AAO ranged from hypoplastic arch in five patients, coarctation two patients, combined four patients and interrupted aortic arch (IAA) two patients. Arterial switch with Lecomte ± VSD repair was performed during cooling, and aortic arch repair was performed under deep hypothermic circulatory arrest (DHCA) (35 ± 14 min at 16.9 ± 0.7 °C). Our preference was to use homograft patch-plasty for arch and direct end-to-side anastomosis for coarctation repair. Aortic-cross-clamp time was 124 ± 24 min and cardiopulmonary bypass (CPB) time 215 ± 84 min. RESULTS: Early survival was 19/22 (86%) up to 30 days without mortality in the second half of our series. Three patients required extracorporeal membrane oxygenation (ECMO) support and renal support was needed in three and preferred permanent pace maker (PPM) implantation in two. Length of stay was 21.9 ± 22.1 days. There was one late death and overall survival was 18/22 (82%) for the follow-up period of 4.8 years (0.2-9.8 years). Eight patients (44%) required re-intervention for re-coarctation. Four patients required right ventricular outflow tract (RVOT)/pulmonary artery re-interventions. At follow-up, there was no requirement for aortic valve replacement, residual VSD closure and no evidence of ventricular dysfunction. CONCLUSIONS: One-stage repair of TGA/DORV and AAO can be performed safely with a good survival rate. Three important lessons that we have learnt are as follows: (1) the subpulmonary VSD may have a perimembraneous component, (2) late re-coarctation is not infrequent and (3) late residual right-sided cardiac lesions remain an issue in complex TGA repair.