Bilateral remote ischemic conditioning in children: A two-center, double-blind, randomized controlled trial in young children undergoing cardiac surgery.

Objective: The study objective was to determine whether adequately delivered bilateral remote ischemic preconditioning is cardioprotective in young children undergoing surgery for 2 common congenital heart defects with or without cyanosis. Methods: We performed a prospective, double-blind, randomized controlled trial at 2 centers in the United Kingdom. Children aged 3 to 36 months undergoing tetralogy of Fallot repair or ventricular septal defect closure were randomized 1:1 to receive bilateral preconditioning or sham intervention. Participants were followed up until hospital discharge or 30 days. The primary outcome was area under the curve for high-sensitivity troponin-T in the first 24 hours after surgery, analyzed by intention-to-treat. Right atrial biopsies were obtained in selected participants. Results: Between October 2016 and December 2020, 120 eligible children were randomized to receive bilateral preconditioning (n = 60) or sham intervention (n = 60). The primary outcome, area under the curve for high-sensitivity troponin-T, was higher in the preconditioning group (mean: 70.0 ± 50.9 µg/L/h, n = 56) than in controls (mean: 55.6 ± 30.1 µg/L/h, n = 58) (mean difference, 13.2 µg/L/h; 95% CI, 0.5-25.8; P = .04). Subgroup analyses did not show a differential treatment effect by oxygen saturations (pinteraction = .25), but there was evidence of a differential effect by underlying defect (pinteraction = .04). Secondary outcomes and myocardial metabolism, quantified in atrial biopsies, were not different between randomized groups. Conclusions: Bilateral remote ischemic preconditioning does not attenuate myocardial injury in children undergoing surgical repair for congenital heart defects, and there was evidence of potential harm in unstented tetralogy of Fallot. The routine use of remote ischemic preconditioning cannot be recommended for myocardial protection during pediatric cardiac surgery.

Congenitally Corrected Transposition of the Great Arteries in Utero: Morphological Spectrum, Outcomes and Pitfalls in Fetal Diagnosis.

Congenitally corrected transposition of the great arteries (ccTGA) is a rare malformation with diverse morphology. We assessed features of fetuses with ccTGA and evaluated neonatal and pediatric outcomes. This was a retrospective review of fetuses with ccTGA at Birmingham Women's and Children's Hospital born from 2005 to 2019. Of thirty-six fetuses identified, six had unavailable prenatal data, one was postnatally diagnosed with isomerism and 29 fetuses were evaluated. ccTGA without associated cardiac lesions was found in 28% (8/29), ccTGA with significant VSD in 31% (9/29), ccTGA with pulmonary obstruction in 24% (7/29) and ccTGA with complex anomalies in 17% (5/29). Tricuspid regurgitation (TR) was observed in 17% (5/29) and heart block (HB) in 10% (3/29) prenatally. Six, that is 21% underwent genetic testing of which one was abnormal. Five extra-cardiac anomalies were reported prenatally and postnatally. Pregnancy was discontinued in five, of which two had moderate TR. There were thirty-one liveborn. Coarctation of the aorta was found in five postnatally but not suspected prenatally. In one, pulmonary stenosis was underestimated; otherwise, prenatal morphology was confirmed. Cardiac interventions were performed in 77% (24/31) liveborn with 39% (12/31) undergoing neonatal intervention. Overall, 6/31 liveborn died including all three with prenatal heart block and one with TR. Estimated survival for all liveborn at 1, 5 and 10 years was 87% (95% CI 76-100%), 83% (95% CI 72-98%) and 80% (95% CI 66-96%) respectively. Accurate prenatal diagnosis of ccTGA is critical for counseling. Early outcomes are favorable with 77% of liveborn undergoing surgery. Fetuses with prenatal diagnosis of complex associated abnormalities, HB and TR appear to do less well.

Which Phenotypes Should We Include in the Hypoplastic Left Heart Syndrome?

The recent special issue of the World Journal for Pediatric and Congenital Heart Surgery devoted to hypoplastic left heart syndrome, and its related anomalies, contained significant information of great clinical relevance. Very little attention, however, was devoted to the integrity of ventricular septum as providing a criterion to distinguish between the phenotypes to be included within the syndrome, as opposed to the related anomalies. In this commentary, we summarize the evidence in support of the notion that the phenotypes to be included within the syndrome can be interpreted on the basis of an acquired disease of fetal life. We suggest that it is the integrity of the ventricular septum that provided the major criterion for the distinction between the lesions making up the syndrome and the related anomalies. The subsets of lesions to be included within the syndrome can then be recognized in terms of the time, subsequent to the closure of the embryonic interventricular communication, at which the left ventricle ceased its growth relative to the remainder of the cardiac components. On this basis, it is possible to recognize the combinations of aortic and mitral atresia, mitral stenosis with aortic atresia, combined mitral and aortic stenosis, and hypoplasia of the left ventricle with commensurate hypoplasia of the aortic and mitral valves; the latter combination now recognized as the hypoplastic left heart complex.

Expert Consensus Statement: Anatomy, Imaging, and Nomenclature of Congenital Aortic Root Malformations.

Over the past 2 decades, several categorizations have been proposed for the abnormalities of the aortic root. These schemes have mostly been devoid of input from specialists of congenital cardiac disease. The aim of this review is to provide a classification, from the perspective of these specialists, based on an understanding of normal and abnormal morphogenesis and anatomy, with emphasis placed on the features of clinical and surgical relevance. We contend that the description of the congenitally malformed aortic root is simplified when approached in a fashion that recognizes the normal root to be made up of 3 leaflets, supported by their own sinuses, with the sinuses themselves separated by the interleaflet triangles. The malformed root, usually found in the setting of 3 sinuses, can also be found with 2 sinuses, and very rarely with 4 sinuses. This permits description of trisinuate, bisinuate, and quadrisinuate variants, respectively. This feature then provides the basis for classification of the anatomical and functional number of leaflets present. By offering standardized terms and definitions, we submit that our classification will be suitable for those working in all cardiac specialties, whether pediatric or adult. It is of equal value in the settings of acquired or congenital cardiac disease. Our recommendations will serve to amend and/or add to the existing International Paediatric and Congenital Cardiac Code, along with the Eleventh iteration of the International Classification of Diseases provided by the World Health Organization.

Outcomes of the arterial switch for transposition during infancy using a standardized approach over 30 years.

OBJECTIVES: The aim of this study was to describe the early and late outcomes of the arterial switch for transposition. METHODS: A single-centre retrospective cohort study was conducted to assess the early and late outcomes of arterial switch performed during infancy using a standardized institutional approach between 1988 and 2018, compared by morphological groups. RESULTS: A total of 749 consecutive patients undergoing arterial switch during infancy were included, 464 (61.9%) with intact septum, 163 (21.8%) with isolated ventricular septal defect and 122 (16.3%) with complex transposition with associated lesions, including 67 (8.9%) with Taussig-Bing anomaly. There were 34 early deaths [4.5%, 95% confidence interval (CI) 3.1-6.1] with only 10 (2.6%) early deaths since 2000. Complex morphology (odds ratio 11.44, 95% CI 4.76-27.43) and intramural coronary artery (odds ratio 5.17, 95% CI 1.61-15.91) were identified as the most important risk factors for 90-day mortality. Overall survival was 92.7% (95% CI 90.8-94.6) at 5 years and 91.9% (95% CI 89.9-94.1) at 20 years; in hospital survivors, there were 15 (2.1%) late deaths during a median follow-up of 13.7 years. Cumulative incidence of surgical or catheter reintervention was 16.0% (95% CI 14.5-17.5) at 5 years and 22.7% (95% CI 21.0-24.0) at 20 years; early and late reinterventions were more common in the complex group, with no difference between the other groups. CONCLUSIONS: Using a standardized approach, the arterial switch can be performed with low early mortality, moderate rates of reintervention and excellent long-term survival. Concomitant lesions were the most important risk factor for early death and were associated with increased risk of late reintervention.

Nomenclature for Pediatric and Congenital Cardiac Care: Unification of Clinical and Administrative Nomenclature - The 2021 International Paediatric and Congenital Cardiac Code (IPCCC) and the Eleventh Revision of the International Classification of Diseases (ICD-11).

Substantial progress has been made in the standardization of nomenclature for paediatric and congenital cardiac care. In 1936, Maude Abbott published her Atlas of Congenital Cardiac Disease, which was the first formal attempt to classify congenital heart disease. The International Paediatric and Congenital Cardiac Code (IPCCC) is now utilized worldwide and has most recently become the paediatric and congenital cardiac component of the Eleventh Revision of the International Classification of Diseases (ICD-11). The most recent publication of the IPCCC was in 2017. This manuscript provides an updated 2021 version of the IPCCC.The International Society for Nomenclature of Paediatric and Congenital Heart Disease (ISNPCHD), in collaboration with the World Health Organization (WHO), developed the paediatric and congenital cardiac nomenclature that is now within the eleventh version of the International Classification of Diseases (ICD-11). This unification of IPCCC and ICD-11 is the IPCCC ICD-11 Nomenclature and is the first time that the clinical nomenclature for paediatric and congenital cardiac care and the administrative nomenclature for paediatric and congenital cardiac care are harmonized. The resultant congenital cardiac component of ICD-11 was increased from 29 congenital cardiac codes in ICD-9 and 73 congenital cardiac codes in ICD-10 to 318 codes submitted by ISNPCHD through 2018 for incorporation into ICD-11. After these 318 terms were incorporated into ICD-11 in 2018, the WHO ICD-11 team added an additional 49 terms, some of which are acceptable legacy terms from ICD-10, while others provide greater granularity than the ISNPCHD thought was originally acceptable. Thus, the total number of paediatric and congenital cardiac terms in ICD-11 is 367. In this manuscript, we describe and review the terminology, hierarchy, and definitions of the IPCCC ICD-11 Nomenclature. This article, therefore, presents a global system of nomenclature for paediatric and congenital cardiac care that unifies clinical and administrative nomenclature.The members of ISNPCHD realize that the nomenclature published in this manuscript will continue to evolve. The version of the IPCCC that was published in 2017 has evolved and changed, and it is now replaced by this 2021 version. In the future, ISNPCHD will again publish updated versions of IPCCC, as IPCCC continues to evolve.

Morphology of vascular ring arch anomalies influences prognosis and management.

OBJECTIVE: This study aimed to explore the anatomical features of aortic arch anomalies associated with vascular rings, hoping to identify those which may increase the risk of symptomatic presentation and surgical intervention. METHODS: This was a retrospective observational study at a single cardiac unit. Individuals diagnosed with an aortic arch anomaly, either isolated or non-isolated, between June 2014 and September 2018 were included. The morphology of the aortic arch was established via analysis of postnatal echocardiography, CT or MRI scans. CT and magnetic resonance studies were evaluated for the presence of a Kommerell diverticulum in those with aberrant vessels. Case notes were reviewed for relevant clinical data. RESULTS: Of those with aberrant subclavian arteries, 24/79 (30.4%) were shown to have a Kommerell diverticulum. Additional forms of congenital heart disease were present in 133/227 (58.6%) individuals. Surgical division of the vascular ring was performed in 30/227 (13.2%), most commonly in the setting of a double aortic arch (70.8%). In those with aberrant subclavian arteries, no children without a Kommerell diverticulum were referred for surgery. In those with a Kommerell diverticulum confirmed on imaging, 11/24 underwent surgery. CONCLUSION: Individuals with a double aortic arch, or an aberrant subclavian artery arising from a Kommerell diverticulum, have the highest requirement for surgical intervention, especially in isolated anomalies. These individuals should remain under monitoring. The subjective nature of symptoms remains problematic. Longitudinal research is required further to understand the natural history of vascular rings and how it links to morphology.

Anatomical Studies of Transposition - An Argument for a Unifying Morphological Classification.

In the setting of transposition, recognition of the variability in the morphology of the outflow tracts and the arterial trunks is essential for surgical repair. Presence of a unifying system for classification would minimize the risk of misunderstanding. We examined an archive of 142 unrepaired hearts with transposition, defined as the combination of concordant atrioventricular and discordant ventriculoarterial connections, combined with the clinical records of 727 patients undergoing the arterial switch procedures. In the setting of usual atrial arrangement, we found the aortic root in leftward or posterior location in up to 5% of our cohorts, making the common term "d-transposition" ambiguous. Variability in the relationship of the trunks was commoner in the setting of deficient ventricular septation, especially when the trunks were side-by-side (14% vs 5.5% when the ventricular septum was intact). Bilateral infundibulums were present in 14% of cases, and bilaterally deficient infundibulums in 3.5%. Both of these findings were more common with deficient ventricular septation. Fibrous continuity between the tricuspid and pulmonary valves was not always seen with perimembranous defects, particularly when there were bilateral infundibulums. Fibrous continuity between the leaflets of the tricuspid and mitral valves, in contrast, proved a unifying characteristic of the perimembranous defect. The combination of concordant atrioventricular and discordant ventriculoarterial connections is best described simply as "transposition," with precision provided when details are given of atrial arrangement and the relationships of the arterial trunks.

Borders as opposed to so-called geography: which should be used to classify isolated ventricular septal defects?

OBJECTIVES: Ventricular septal defects can be classified according to their borders or according to the fashion in which they open to the right ventricle, so-called geography. As yet, there is no consensus as to how they should be classified. In an attempt to achieve agreement, the International Society for Nomenclature of Congenital and Paediatric Heart Disease, in 2018, proposed a system incorporating both approaches. We have assessed the subjectivity of their suggested terms hoping to determine their suitability in the desired universal system for classification. METHODS: We examined 212 specimens held in the archive of Birmingham Women's and Children's Hospital. Each defect was described by 3 independent examiners on the basis of borders and their relationship to the landmarks of the right ventricle. The interobserver agreement was then calculated using Fleiss' method. RESULTS: Calculations to assess interobserver agreement showed that the examiners were more likely to agree on the borders of the defects than their so-called geography (κ = 0.804 vs κ = 0.518). The landmarks of the right ventricle proved to be highly variable such that the application of 'geographic' terms to hearts with perimembranous defects proved particularly challenging. CONCLUSIONS: Interobserver agreement is lower when using terms based on 'geography' as opposed to borders. Whilst providing important morphological detail, the terms based on right ventricular landmarks are highly subjective. They should not be prioritized in a universal system of classification. Instead, the defects can be classified simply by using 'perimembranous', 'muscular', or 'doubly committed and juxta-arterial' as first-order terms.

An Unusual Combination of Double Inlet Left Ventricle With Discordant Ventriculoarterial Connections and Bilateral Arterial Ducts.

The presence of bilaterally persistent arterial ducts is an uncommon abnormality. Here, we describe the anatomy and successful management of an unusual patient with bilateral ducts in the setting of double inlet left ventricle, discordant ventriculoarterial connections, aortic atresia, and a severely hypoplastic and serpentine aortic arch.

Double Outlet Right Ventricle With Right-Sided Aorta From the Left-Sided Morphologically Right Ventricle in the Setting of Discordant Atrioventricular Connections.

We describe the anatomic findings in a 2-year-old patient with double outlet right ventricle with right-sided aorta in the setting of usual atrial arrangement and discordant atrioventricular connections, making comparison with a specimen from the pathological archive of the Birmingham Children's Hospital in the United Kingdom having this rare combination of anatomic features. We discuss the challenges involved in diagnosis and management.

Tetralogy of Fallot: morphological variations and implications for surgical repair.

OBJECTIVES: Tetralogy of Fallot is characterized by anterocephalad deviation of the outlet septum, along with abnormal septoparietal trabeculations, which lead to subpulmonary infundibular stenosis. Archives of retained hearts are an important resource for improving our understanding of congenital heart defects and their morphological variability. This study aims to define variations in aortic override, coronary arterial patterns and ventricular septal defects in tetralogy of Fallot as observed in a morphological archive, highlighting implications for surgical management. METHODS: The Birmingham Children's Hospital archive contains 211 hearts with tetralogy of Fallot, of which 164 were analysed [69 (42.1%) unrepaired and 95 (57.9%) operated specimens]. A detailed morphological and geometric analysis was performed using a rigorous 5-layer review process. RESULTS: Anomalies were observed in the orifices, origins and course of the coronary arteries: 20 hearts (13.0%) had more than 2 orifices and 3 hearts (1.9%) had a single orifice. In 7 hearts (4.3%), a coronary artery crossed the right ventricular outflow tract. The extent of aortic override ranged from 31.0% to 100% (median of 59.5%). The ventricular septal defect was most often perimembranous (139, 84.8%), but we also found muscular (14, 8.5%), atrioventricular (7, 4.3%) and doubly committed juxta-arterial (2, 1.2%) variants. CONCLUSIONS: Anatomical variations are common and can impact surgical management. Anomalous coronary arteries may require a conduit rather than a transannular patch. Variability in aortic override determines the size of patch used to baffle blood to the aorta. The type of ventricular septal defect affects patch closure and the risk of postoperative conduction defects.

Classification of Ventricular Septal Defects for the Eleventh Iteration of the International Classification of Diseases-Striving for Consensus: A Report From the International Society for Nomenclature of Paediatric and Congenital Heart Disease.

The definition and classification of ventricular septal defects have been fraught with controversy. The International Society for Nomenclature of Paediatric and Congenital Heart Disease is a group of international specialists in pediatric cardiology, cardiac surgery, cardiac morphology, and cardiac pathology that has met annually for the past 9 years in an effort to unify by consensus the divergent approaches to describe ventricular septal defects. These efforts have culminated in acceptance of the classification system by the World Health Organization into the 11th Iteration of the International Classification of Diseases. The scheme to categorize a ventricular septal defect uses both its location and the structures along its borders, thereby bridging the two most popular and disparate classification approaches and providing a common language for describing each phenotype. Although the first-order terms are based on the geographic categories of central perimembranous, inlet, trabecular muscular, and outlet defects, inlet and outlet defects are further characterized by descriptors that incorporate the borders of the defect, namely the perimembranous, muscular, and juxta-arterial types. The Society recognizes that it is equally valid to classify these defects by geography or borders, so the emphasis in this system is on the second-order terms that incorporate both geography and borders to describe each phenotype. The unified terminology should help the medical community describe with better precision all types of ventricular septal defects.

The importance of age and weight on cavopulmonary shunt (stage II) outcomes after the Norwood procedure: Planned versus unplanned surgery.

OBJECTIVE: The study objective was to evaluate the outcomes of the cavopulmonary shunt after the Norwood procedure with a particular focus on age, weight, and whether surgery was planned or expedited by clinical findings. METHODS: We studied 297 consecutive patients with hypoplastic left heart syndrome undergoing the cavopulmonary shunt operation between 2002 and 2014. All patients underwent the Norwood procedure with a right ventricle to pulmonary artery conduit. Unplanned surgery was any situation in which surgery was expedited because of refractory desaturation, angiographic findings, or failure to discharge. RESULTS: There were 222 planned and 75 unplanned procedures. Planned surgery was performed at median of 5.4 (4.6-6.3) months compared with 3.9 (3.3-5.0) months for the unplanned group (P < .001). Early mortality was 1.3% in the planned group compared with 5.3% in the unplanned group (P = .07). At 5 and 10 years, survival was significantly worse in the unplanned group (73% vs 85%, P = .03). On multivariable analysis, atrioventricular valve regurgitation, the need for atrioventricular valve repair, and impaired ventricular function (graded composite variable) were the predominant risk factors (hazard ratio [HR], 7.49), with an incremental risk within these 3 variables. The planned versus unplanned nature of the surgery was less significant (HR, 1.75) because it was eclipsed by ventricular function and tricuspid intervention. Additional factors were aortic atresia/mitral atresia subgroup (HR, 2.07) and somatic growth (z-score weight) at the time of surgery (HR, 0.60 for z-score change from -2.54 to -1.16). Age and weight at surgery were not significant risk factors for survival. Interstage interventions on the aortic arch or right ventricle to pulmonary artery conduit did not adversely influence outcomes. CONCLUSIONS: The ventricular function, atrioventricular valve regurgitation, and need for tricuspid intervention were the primary risk factors for survival after the cavopulmonary shunt operation. Although unplanned surgery is associated with poor outcomes, this is related to the primary risk factors and not the timing or age of the patient. Somatic growth also has a significant influence on survival. Thus, an earlier cavopulmonary shunt operation is safe in infants who are thriving, but those with poor weight gain are at higher risk.

Truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage: a unique form of univentricular heart.

We present the first reported case of truncus arteriosus, tricuspid atresia and partial anomalous pulmonary venous drainage into a retro-aortic innominate vein in a neonate in whom successful palliation was performed using a two-staged procedure. An early repair after presentation along with a re-routing of anomalous pulmonary venous drainage at the time of the second stage should be undertaken to optimize the patient's haemodynamic status prior to a Fontan completion.

Toward predictable repair of regurgitant aortic valves: a systematic morphology-directed approach to bicommissural repair.

OBJECTIVES: Our purpose was to investigate a new approach to bicommissural repair of regurgitant aortic valves. BACKGROUND: Repair of regurgitant aortic valves is not widely accepted, but interest is increasing, particularly for bicuspid valves. We hypothesize that a systematic, segmental approach to morphology and corresponding morphology-directed repair will improve decision making and success. METHODS: From December 2001 to July 2007, a systematic surgical approach to valve analysis and bicommissural repair was applied prospectively to 63 consecutive patients with pure aortic valve regurgitation, mean age 40 +/- 12 years. Cusp, commissure, and root morphologies were analyzed sequentially by direct inspection. Each abnormality was corrected by corresponding morphology-directed repair procedures. Retrospectively, 2 echocardiographic indexes--of tissue pliability (change in systolic to diastolic area) and coaptation deficiency (conjoint and reference cusp heights vs. "annulus" diameter)--were developed to evaluate repairability. RESULTS: Forty-two (67%) valves were repaired and 21 (33%) replaced. Regurgitation was related primarily to cusp (prolapse, restriction) and commissure (splaying) morphology; root pathology was less important. Morphology-directed repair included cusp maneuvers in all, commissural maneuvers in 71%, and root procedures in 33%. Restriction and cusp tissue deficiency limited repairability. Echocardiography reflected this in greater tissue pliability of successfully repaired valves compared with replaced ones (conjoint cusp 61 +/- 16% vs. 34 +/- 17%; reference cusp 65 +/- 16% vs. 42 +/- 16%; p = 0.0001) and less coaptation deficiency (1.06 +/- 0.24 for repaired and 1.27 +/- 0.19 for replaced valves; p = 0.002). CONCLUSIONS: Systematic segmental analysis of morphology and a logical morphology-directed surgical approach facilitate aortic valve repair. Initial application of this paradigm suggests sufficient mobile cusp tissue is a key determinant of repairability.

Repair of complete atrioventricular septal defects in patients weighing less than 5 kg.

BACKGROUND: The aim of this study was to evaluate the impact of weight less than 5 kg at operation on mortality and morbidity in patients with atrioventricular septal defect (AVSDc) undergoing total correction. METHODS: Between January 1990 and December 2002, 190 consecutive patients with AVSDc underwent total biventricular correction. They were divided into two groups: group I (n = 64 patients weighing < 5 kg) and group II (n = 126 patients weighing > 5 kg). Associated major cardiac malformations were found in 49 (25.8%) patients. Associated left atrioventricular valve (LAVV) malformations were found in 35 (18.4%) patients. The mean follow-up time was 4.1 +/- 2.9 years (range 2 months-10.7 years). RESULTS: The in-hospital mortality in group I was 7.8% (5 patients) versus 8.7% (11 patients) in group II (p = 0.95). Major associated cardiac malformations (p < 0.001) and pulmonary hypertension (p = 0.006) were found to be strong predictors for poor postoperative survival. At discharge the mean LAVVR grade in group I was 1.45 +/- 1.2 versus 1.2 +/- 1 in group II (p = 0.13). The actuarial overall survival rates at 1, 3, 5, and 7 years were 96.5%, 92.5%, 91.5%, and 89% respectively and the actuarial overall reoperation free survival rates at 1, 3, 5, and 7 years were 95%, 87%, 84%, and 73%. Twenty-three patients underwent reoperation due to severe left atrioventricular valve regurgitation (LAVVR). Strong predictors for overall reoperation free survival were the operation year before 1995 (p < 0.001), postoperative LAVVR greater than or equal to 2 (p = 0.006), major associated cardiac malformations (p = 0.00034), associated LAVV malformations (p = 0.0044), and non or partial LAVV cleft closure (p = 0.012). The actuarial survival rates between patients weighing less than 5 kg versus patients weighing more than 5 kg were similar (p = 0.51); instead the overall reoperation free survival was significantly lower in patients weighing less than 5 kg (p = 0.022) according to the log-rank test. Weight less than 5 kg (p = 0.023, beta = -0.6) was one of the predictors for reoperation due to severe LAVVR in this series. CONCLUSIONS: We may conclude that in the current era repair of AVSDc can be carried out successfully in patients less than 5 kg, however, weight less than 5 kg at initial complete repair seems to be a predictor for late reoperation due to LAVVR. Suture separation at the cleft site or between the leaflets of the newly created mitral valve and the patch remain the main causes of postoperative LAVVR in patients weighing less than 5 kg.

Anomalous origin of the right pulmonary artery from the ascending aorta.

OBJECTIVES: The aim was to review our experience with the surgical repair of the anomalous origin of the right pulmonary artery (AORPA) from the aorta. MATERIALS AND METHOD: Between January 1991 and March 2001, five patients with AORPA underwent surgical correction. One patient presented isolated AOPA from the aorta. Implantation of the anomalous PA to the main PA trunk was performed by (1). direct anatomosis employing an autologous pericardial patch in two patients; (2). using an aortic flap in two patients with AORPA; and (3). using an aortic and pulmonary flaps in another patient. The mean follow-up time was 27 months. RESULTS: One patient died due to progressive heart failure unresponsive to inotropic support. Early postoperative pulmonary hypertension crisis was identified in patient 4, that was managed by intravenous prostacyclin. The same patient necessitated mechanical ventilation for 11 days. The mean residual gradient for all survivors was 9.5 +/- 4 mmHg. The postoperative Tc-99 m scintigraphy demonstrated 72 +/- 4.5(%) lung perfusion. At follow-up the survival was 100%. None of the followed patients required reoperation. CONCLUSION: The AORPA from the aorta is a rare but important entity, necessitating a scrupulous preoperative and intraoperative evaluation. The techniques employing autologous tissues for enlarging and lengthening the AORPA seem to be associated with better results in terms of postoperative restenosis.

Total correction of complete atrioventricular septal defect with tetralogy of Fallot.

BACKGROUND AND AIM OF THE STUDY: The authors' experience in surgical management of tetralogy of Fallot in association with complete atrioventricular septal defect (CAVSD-TOF) is reported. METHODS: Between January 1990 and September 2001, 146 children with CAVSD underwent complete correction. ASD closure was performed using a one-patch technique, with autologous pericardial patch. Seventeen patients presented with CAVSD-TOF, of whom nine (53%) had undergone previous palliation. Mean age at repair was 2.9 +/- 1.9 years. The mean gradient across the right ventricular outflow tract (RVOT) was 63 +/- 16 mmHg. Six patients (35%) required a transannular patch. RESULTS: Overall in-hospital mortality was 10%. Among patients with CAVSD-TOF, three died in hospital (18%); causes of death were progressive heart failure (n = 2) and multiple organ failure (n = 1). Two patients required mediastinal exploration due to significant bleeding, and two late reoperation for severe left atrioventricular valve regurgitation after intracardiac repair. Mean follow up was 36 +/- 34 months. All patients survived and are currently in NYHA class I or II. At follow up, mean gradient across the RVOT was 17 +/- 6 mmHg, significantly less than the preoperative value (p < 0.001). CONCLUSION: Complete repair in patients with CAVSD-TOF offers acceptable early and mid-term outcome in terms of mortality, morbidity and reoperation rate. Palliation prior to repair may be preferred in cases with small pulmonary arteries, or in severely cyanotic neonates. The RVOT should be managed as for isolated TOF, but a transatrial-transpulmonary approach is the surgery of choice.