Accuracy and impact of prenatal diagnosis of common arterial trunk.

OBJECTIVES: Outcome of common arterial trunk (CAT) depends mainly on truncal valve function, presence of coronary artery abnormalities and presence of interrupted aortic arch. The main objective of this study was to evaluate the accuracy of prenatal diagnosis of CAT by analyzing prenatal vs postnatal assessment of: (1) anatomic subtypes and (2) truncal valve function. The secondary objective was to assess the potential impact of prenatal diagnosis of CAT on postnatal mortality and morbidity by comparing prenatally vs postnatally diagnosed patients. METHODS: This was a retrospective analysis of all CAT patients diagnosed either prenatally, with postnatal or fetopsy confirmation, or postnatally, from 2011 to 2019 in a single tertiary center. Cohen's kappa statistic was used to evaluate agreement between pre- and postnatal assessment of anatomic subtypes according to Van Praagh and of truncal valve function. Mortality and morbidity variables were compared between prenatally vs postnatally diagnosed CAT patients. RESULTS: A total of 84 patients (62 liveborn with prenatal diagnosis, 16 liveborn with postnatal diagnosis and six terminations of pregnancy with fetopsy) met the inclusion criteria. The accuracy of prenatal diagnosis of CAT anatomic subtype was 80.3%, and prenatal and postnatal concordance for subtype diagnosis was only moderate (κ = 0.43), with no patient with CAT Type A3 (0/4) and only half of patients with CAT Type A4 (8/17) being diagnosed prenatally. Fetal evaluation of truncal valve function underestimated the presence (no agreement; κ = 0.09) and severity (slight agreement; κ = 0.19) of insufficiency. However, four of five cases of postnatally confirmed significant truncal valve stenosis were diagnosed prenatally, with fair agreement for both presence and severity of stenosis (κ = 0.38 and 0.24, respectively). Mortality was comparable in patients with and those without prenatal diagnosis (log-rank P = 0.87). CAT patients with fetal diagnosis underwent earlier intervention (P < 0.001), had shorter intubation time (P = 0.047) and shorter global hospital stay (P = 0.01). CONCLUSIONS: The accuracy of prenatal diagnosis of CAT is insufficient to tailor neonatal management and to predict outcome. Fetal assessment of truncal valve dysfunction appears unreliable due to perinatal transition. Improvement is necessary in the fetal diagnosis of anatomic subtypes of CAT requiring postnatal prostaglandin infusion. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Origin of congenital coronary arterio-ventricular fistulae from anomalous epicardial and myocardial development.

Coronary Artery Fistulae (CAFs) are cardiac congenital anomalies consisting of an abnormal communication of a coronary artery with either a cardiac chamber or another cardiac vessel. In humans, these congenital anomalies can lead to complications such as myocardial hypertrophy, endocarditis, heart dilatation, and failure. Unfortunately, despite their clinical relevance, the aetiology of CAFs remains unknown. In this work, we have used two different species (mouse and avian embryos) to experimentally model CAFs morphogenesis. Both conditional Itga4 (alpha 4 integrin) epicardial deletion in mice and cryocauterisation of chick embryonic hearts disrupted epicardial development and ventricular wall growth, two essential events in coronary embryogenesis. Our results suggest that myocardial discontinuities in the embryonic ventricular wall promote the early contact of the endocardium with epicardial-derived coronary progenitors at the cardiac surface, leading to ventricular endocardial extrusion, precocious differentiation of coronary smooth muscle cells, and the formation of pouch-like aberrant coronary-like structures in direct connection with the ventricular lumen. The structure of these CAF-like anomalies was compared with histopathological data from a human CAF. Our results provide relevant information for the early diagnosis of these congenital anomalies and the molecular mechanisms that regulate their embryogenesis.

Evaluation of septal insertion of atrioventricular valves in fetuses by postmortem 4.7 Tesla cardiac MRI: A feasibility study.

PURPOSE: The purpose of this study was to compare non-invasive high-spatial-resolution postmortem cardiac magnetic resonance imaging (MRI) and autopsy findings for evaluating the septal insertion of atrioventricular valves in fetuses. MATERIALS AND METHODS: Five fetal heart specimens including two normal hearts, one heart with complete atrioventricular septal defect (AVSD) and two hearts with linear insertion of atrioventricular valves (LIAVV; gestational age 17 to 34 weeks) were studied with cardiac MRI using a 4.7 T MRI scanner without sample preparation. Three (3D) and two-dimensional (2D) turbo-RARE (rapid imaging with refocused echoes) sequences in four-chamber and left-ventricular long-axis planes were obtained with a minimal isotropic/in-plane resolution of 156µm. Nonparametric tests were performed to compare the distance between insertions of medial leaflets of the atrioventricular valves and the inlet/outlet distance ratio between MRI and autopsy findings in normal, complete AVSD and with linear insertion of atrioventricular valves (LIAVV) fetal hearts. RESULTS: Despite apparent differences between LIAVV/normal hearts, no significant differences were found between differential insertion of medial leaflets and inlet/outlet distance ratios with both techniques. Very good to excellent reliability between both techniques was found for differential insertion (ICC: 87.2%; 95% CI: -21.7%, 99.1%) (P=0.963) and inlet/outlet distance ratio (ICC 98.3%; 95%CI: 85.2%, 99.8%) (P=0.537) measurements. CONCLUSION: Postmortem cardiac MRI could replace autopsy for assessing normal or abnormal septal insertion of atrioventricular valves in fetuses without requiring specific preparation of the heart.

Congenital mitral stenosis with or without associated defects: An evolving surgical strategy.

BACKGROUND: Congenital mitral stenosis (CMS) remains a surgical challenge, particularly when it is associated with other heart defects. As in other groups of heart defects, there is a trend toward early single-stage complete repair, but the optimal surgical approach remains unanswered. METHODS AND RESULTS: This study was designed to analyze the evolution of surgical strategies in patients with CMS and associated defects through single-stage and staged repair. Between 1980 and 1999, 72 children were operated on for congenital heart defects, including CMS. Preoperative transmitral gradient was 12.6+/-7 mm Hg. Preoperatively, all the patients were NYHA class III to IV. Thirteen patients had an isolated CMS; in 59, it was associated with other heart defects, mainly ventricular septal defect (n=28) or multilevel left ventricular obstruction (n=41). In this group of patients, 33 had a staged approach, and 26 had a single-stage approach. Early mortality was 12.5% (9 patients). There were no deaths in the isolated CMS and single-stage repair groups. Logistic regression revealed that early mortality was influenced by association with left ventricular outflow tract obstruction (P:<0.001) and by use of a staged approach (P:<0.01). There was no late mortality in isolated CMS; there were 2 late deaths in the group of single-stage repair and 6 late deaths in the staged approach group (P:<0.01). Reoperation was required in 24 patients, mainly for residual mitral valve dysfunction or residual left ventricular outflow tract obstruction. Including the reoperations, 10 patients received a prosthetic mitral valve. At 15 years after surgery, survival was 69.6+/-7.5%, freedom from reoperation was 70.8+/-6.3%, and freedom from mitral valve replacement was 69+/-6%. CONCLUSIONS: Surgery for isolated CMS gives excellent early and long-term results. In patients with associated heart defects, a single-stage operation seems superior to a staged approach. Mitral valve replacement in this category of patients should be reserved as a salvage procedure.

Ventricular arrhythmias after correction of ventricular septal defects: importance of surgical approach.

To compare the prevalence of conduction disturbances and ventricular arrhythmias in cases of postoperative ventricular septal defect, 100 patients (50 with repair by right atriotomy, group 1; and 50 with repair by right ventriculotomy, group 2) underwent complete evaluation including an electrocardiogram (ECG) and 24 h ambulatory ECG monitoring. The two groups were comparable except for a shorter follow-up duration (7 +/- 3 versus 12.4 +/- 7 years) and a younger age at evaluation (12.4 +/- 5 versus 16.9 +/- 7 years) in group 1. Complete right bundle branch block was less frequent in group 1 than in group 2 (20% versus 50%, p less than 0.05) but three of the four patients with complete atrioventricular (AV) block detected on ambulatory monitoring were in group 1. Six patients had significant supraventricular arrhythmias, all well tolerated. Ambulatory monitoring revealed significant ventricular arrhythmias (modified Lown grade 2 or higher) in 39 patients, with a lower prevalence in group 1 (30% versus 48%, p = 0.05). No correlation was found between prevalence of ventricular arrhythmias and right ventricular systolic pressure, cardiopulmonary bypass duration, presence of a synthetic patch, previous pulmonary artery banding, presence of complete right bundle branch block and cardiomegaly on chest X-ray film. Prevalence of ventricular arrhythmias increased with follow-up duration, age at evaluation and age at surgery. These were always well tolerated and did not warrant treatment. Thus, right atriotomy reduces the prevalence of right bundle branch block but does not prevent late AV block. Ventricular arrhythmias are frequent after surgical closure of ventricular septal defect whatever the surgical approach and their prevalence is not statistically different from that in postoperative tetralogy of Fallot.(ABSTRACT TRUNCATED AT 250 WORDS)

[Surgical repair of atrial septal defect in 1011 consecutive patients]. / Traitement chirurgical des communications interauriculaires chez 1011 patients consécutifs. Incidence des complications postopératoires précoces.

The immediate postoperative complications of 1011 consecutive patients undergoing surgical repair of atrial septal defects between 1980 and 1998 at Marie Lannelongue Hospital were analysedwith the ultimate objective of comparing their incidence with that of percutaneous closure. Five patients died (0.49%) of low cardiac output (N=3), pulmonary oedema (N=1) or pulmonary hypertension (N=1). A total of 356 patients (35.2%) had 448 postoperative complications. There were 77% minor and 23% major complications. The minor complications included arrhythmias and conduction defects (N=130), respiratory complications (N=90) and pericardial effusions (N=64). The main major complications were cardiac failure (N=27), cardiac tamponade (N=13), neurological complications (N=8) and reoperation (N=28). Of the survivors, 95.6% of patients were discharged from hospital with no residual problem and 41 (4.4%) had sequellae: arrhythmias (N=29, including one pacemaker implantation), neurological complications (N=4), acquired mitral regurgitation (N=2), phrenic nerve paralysis (N=1) and minimal residual shunt (N=10). Factors correlated with surgical morbidity were age, the severity of pulmonary hypertension, the type of atrial septal defect (less morbidity with ostium secundum defects), the presence of associated malformations, the surgical approach (less morbidity with the right postero-lateral thoracic approach). This study demonstrated the incompressible risk of open heart cardiac surgery. It will form a basis for a comparative study of the two modern methods of treating atrial septal defect: surgical and percutaneous closure.

Frequency and prognosis of arrhythmias after operative "correction" of tetralogy of Fallot.

Two hundred twenty-four consecutive patients operated on for tetralogy of Fallot were followed from 1 to 28 years (mean 11). Mean age at surgery was 5.3 years (range 1 to 14). Postoperative right ventricular systolic pressure was 60 mm Hg in 19 of 213 patients (9%). Fourteen patients (6%) had ventricular premature complexes on surface electrocardiograms. Seventy-nine patients underwent treadmill exercise tests, and ventricular premature complexes were induced in 17 (22%). Twenty-four-hour ambulatory monitoring in 92 patients demonstrated significant ventricular arrhythmias (greater than or equal to grade 2 of the Lown classification) in 41 (45%). The frequency of ventricular arrhythmias correlated with length of follow-up and duration of cardiopulmonary bypass. No correlation was found with age at surgery, postoperative right ventricular systolic pressure and importance of conduction defects on electrocardiogram. There were no sudden or unexpected deaths during follow-up.

Transposition of the great arteries [S,D,L]. Pathologic anatomy, diagnosis, and surgical management of a newly recognized complex.

The transposition of the great arteries [S,D,L] complex is delineated for the first time from the anatomic, diagnostic, and surgical standpoints in this study of 26 cases: 16 surgical and 10 postmortem. Transposition of the great arteries with situs solitus of the viscera and atria (S), D-loop ventricles (D), and L-transposition (L) was characterized by six additional interrelated anomalies that largely determined surgical management: (1) ventricular septal defect, usually conoventricular, in 96%; (2) malalignment of the conal septum, typically leftward and posteriorly, in 80%; (3) right ventricular hypoplasia in 50%; (4) pulmonary outflow tract stenosis in 27%; (5) ventricular malposition, such as superoinferior ventricles, in 23%; and (6) absent left coronary ostium resulting in "single" right coronary artery in 23%. Complete surgical repair was done in 81% of the surgical patients with a 12.5% hospital mortality rate and no late deaths. When there was no pulmonary outflow tract stenosis and intracardiac anatomy was uncomplicated, we undertook anatomic repair before 1 month of age. However, when pulmonary outflow tract stenosis coexisted, complete repair was deferred until after age 1 year, our currently preferred operation being the REV procedure (réparation a l'etage ventriculaire). When complex intracardiac anatomy precluded biventricular repair, a palliative procedure was performed in 19% without mortality. Hence, this experience indicates that surgical management of patients with the transposition of the great arteries [S,D,L] complex is feasible.

Surgical treatment of subaortic stenosis after biventricular repair of double-outlet right ventricle.

Out of 180 patients who underwent biventricular repair of double-outlet right ventricle between 1980 and 1995, 9 (5%) required reoperation because of subaortic stenosis. Two other patients who initially underwent operation elsewhere underwent reoperation at our institution because of subaortic stenosis. The median age at biventricular repair was 4 months. Repair consisted of tunnel construction from the left ventricle to the aorta in nine patients; the remaining two patients received an arterial switch operation with ventricular septal defect closure. Subaortic stenosis developed with time: the mean postoperative left ventricle-to-aorta gradient after repair was 10 +/- 19 mm Hg (range, 0 to 50 mm Hg) and became 84 +/- 27 mm Hg (range, 40 to 124 mm Hg) in a mean delay of 45 +/- 66 months (range, 1 to 213 months). At reoperation, the obstruction was caused by the protrusion of the inferior rim of the ventricular septal defect into the left ventricular outflow tract associated with subaortic hypertrophied muscle and membrane. The 11 patients underwent 15 reoperations. Surgical technique consisted of an extended septoplasty in 6 reoperations. In this technique an incision was made in the septal patch and was extended into the muscle toward the apex until a large opening of the left ventricular outflow pathway was obtained. A new patch was then secured to streamline the left ventricular outflow tract. None of the patients who underwent extended septoplasty had to undergo reoperation. There were no early or late deaths. At 115 +/- 85 months after biventricular repair, all patients were in New York Heart Association functional class I or II and the mean postoperative left ventricle-to-aorta gradient was 20 +/- 24 mm Hg (range, 0 to 60 mm Hg). We conclude that after biventricular repair of double-outlet right ventricle, the subaortic region is at risk for the development of stenosis. Surgical treatment adapted to the anatomy of the obstruction can offer good early and midterm results. It seems that an aggressive approach by an extended septoplasty avoids multiple reoperations.

Surgically created double-orifice left atrioventricular valve: a valve-sparing repair in selected atrioventricular septal defects.

OBJECTIVES: Some features of the left atrioventricular valve (large mural leaflet, dystrophic tissue) represent a challenge for repair of atrioventricular septal defects without postoperative regurgitation. A retrospective study was conducted to evaluate the results of surgically creating a double-orifice left atrioventricular valve in such circumstances. Clinical results were analyzed according to valvular and subvalvular left atrioventricular valve measurements in pathologic specimens with atrioventricular septal defects. METHODS: Among 157 patients operated on for atrioventricular septal defect since October 1989, 10 patients underwent primary repair (n = 8) or reoperation (n = 2) by this procedure. Median age at repair was 3.3 years (0.1-33 years). Anatomic types were complete (n = 3), intermediate (n = 5), and partial (n = 2). Preoperative moderate to severe left atrioventricular valve regurgitation was present in 6 patients. After the repair (two-patch technique in complete atrioventricular septal defect, cleft closed in each case), these 10 patients were found to have moderate to severe residual regurgitation not amenable to repair by annuloplasty. The top edge of the mural leaflet was anchored to the facing free edge of the cleft. RESULTS: No hospital death or morbidity was observed. Left atrioventricular valve regurgitation was absent or trivial (8 patients) and mild (2 patients). Color-coded echocardiography did not show significant left atrioventricular valve stenosis. The mean diastolic pressure gradient across the left atrioventricular valve was 3.2 +/- 1.1 mm Hg (1.4-4.5 mm Hg). At a median follow-up of 72 months (6-91 months), there was 1 late death, unrelated to left atrioventricular valve malfunction, due to pulmonary vascular obstructive disease. Left atrioventricular valve regurgitation did not increase over time, except in 1 patient in whom regurgitation recently progressed from mild to moderate. At rest, the mean diastolic pressure gradient across the left atrioventricular valve was 3.8 +/- 2.9 mm Hg (1.5-11.2 mm Hg). One child had an early moderate stenosis without pulmonary hypertension. Studies on pathologic specimens (n = 34) indicated that long chordal lengths and large mural leaflet size are essential independent anatomic features to assess its feasibility. CONCLUSIONS: Surgical creation of a double-orifice left atrioventricular valve is an effective additional procedure for repair of atypical cases of atrioventricular septal defect. The operation may decrease the need for reoperation or left atrioventricular valve replacement.

Surgical approaches for double-outlet right ventricle or transposition of the great arteries associated with straddling atrioventricular valves.

The surgical management of patients with double-outlet right ventricle or transposition of the great arteries and straddling atrioventricular valves remains a subject of controversy. Biventricular repair has theoretic advantages because it establishes normal anatomy and physiology. In some instances, however, it seems to carry too high operative risk, and a univentricular heart repair is preferred. Since 1984, we have operated on 34 patients with double-outlet right ventricle (n = 15) or transposition of the great arteries (n = 19) with isolated straddling tricuspid valve (n = 17), isolated straddling mitral valve (n = 9), both mitral and tricuspid straddling (n = 2), or abnormal insertion of tricuspid (n = 7) or mitral (n = 2) chordae in the left ventricular outlet, precluding an adequate tunnel construction. Straddling was categorized according to the location of the papillary muscle insertion in the opposite ventricular chamber: type A, on the edge of the ventricular septal defect (n = 14); type B, on the opposite side of the ventricular septum away from the edge of the defect (n = 8); type C, on the free wall of the opposite ventricular chamber (n = 8). Abnormal chordal insertions were classified according to the location of their attachments around the edges of the defect. Three types of chordal distribution were identified: on the aortic conus, on the pulmonary conus crossing the ventricular septal defect, or around the defect closing it like a curtain. All but three patients had two ventricles of adequate size. Sixteen patients underwent palliation. Median age at the definitive operation was 6.5 months (range 1 to 130 months). Thirty patients underwent a biventricular repair and four had a univentricular repair. Biventricular repair was achieved by an arterial switch operation in 18 patients and by tunnel construction from the left ventricle to the aorta in 12. In isolated straddling of types A and B, the ventricular septal defect was closed by adjusting the septal patch on the ventricular side above the straddled papillary muscle. In type C, the patch was sewn over the papillary muscle by applying it on the septum. In double straddling, the ventricular septum was incised between the two papillary muscles, and an ellipsoid patch was used to reconstruct the septal defect, directing each subvalvular apparatus into its own ventricular chamber. When the abnormal chordae in the left outflow tract inserted on the aortic or pulmonary conus, the conus was incised and tailored to make a flap, leaving an unobstructed left ventricular outflow tract. In two patients the subvalvular apparatus was resected and reattached to the patch. Curtainlike chordae were a contraindication to biventricular repair in double-outlet right ventricle but not in transposition. There were four early deaths and one late death, all occurring in the group having biventricular repair. Death was due to myocardial ischemia (n = 1), right ventricular hypoplasia (n = 1), pulmonary hypertension (n = 1), and residual subaortic stenosis (n = 1). Two patients had moderate to severe postoperative atrioventricular valve incompetence, caused by a cleft in the mitral valve in one patient. Three patients were reoperated on for subaortic stenosis (n = 1), pulmonary stenosis (n = 1), and mitral regurgitation (n = 1). Mean follow-up of 30.7 +/- 19.4 months was achieved in the survivors. All but one patient (univentricular repair) were in New York Heart Association class I, without atrioventricular valve incompetence. Actuarial survival at 4 years was 85.3% +/- 3%. We conclude that straddling or abnormal distribution of chordae tendineae of the atrioventricular valves does not preclude biventricular repair in double-outlet right ventricle or transposition of the great arteries provided that the ventricles are of adequate size. Curtainlike abnormal tricuspid chordae remain a contraindication to biventricular repair in double-outlet right ventricle.

Adult heart transplantation: adverse role of chronic alcoholism in donors on early graft function.

Because of the increasing shortage of heart donors, selection criteria have been gradually extended. The purpose of this study was to determine the donor-related factors implied in early graft dysfunction and to define new selection criteria. The 70 consecutive adult patients who underwent heart transplantation in our institution between January 1988 and February 1992 were retrospectively studied. Mean donor age was 38 +/- 11 years (10 donors were more than 50 years of age; two donors were more than 60 years of age). Mean ischemic time was 130 +/- 39 minutes. An important proportion of donors (20%) had a history of chronic alcoholism. Thirteen patients experienced immediate graft dysfunction; five of them died within the first operative month. The different parameters studied, which were found to have no significant influence on the early graft function, were the age of the donor, the duration of inotropic support and the dose administered, a relative hemodynamic instability, resuscitation maneuvers, chest trauma, and weight mismatch between donor and recipient. Ischemic time was significantly longer in patients who died of cardiac dysfunction (p < 0.05). Chronic alcoholism in the donor was a very detrimental factor: 54% of patients who had early graft dysfunction versus only 12% of patients who had immediate normal graft function had received a graft from an alcoholic donor (p = 0.003). Excluding such alcoholic donors or reserving them for critically-ill recipients, with an increased risk of early graft dysfunction would be preferable.

Prevention of unbalanced lung perfusion and atrial arrhythmias after the lateral tunnel operation.

BACKGROUND: The lateral tunnel operation has become increasingly popular with pediatric cardiac surgeons, as it is technically reproducible, is relatively easy to perform, and can be used in a variety of patients with single-ventricle physiology. The main drawbacks of the original operation are uneven blood flow distribution to the lungs and increasing incidence of supraventricular arrhythmias over time. METHODS: In 1988, we modified this technique by avoiding narrowing of the tunnel at the superior vena cava-atrial junction, avoiding incorporation of the crista terminalis in the baffle suture line, and minimizing damage to the sinoatrial node. Between 1988 and 1995, 19 patients underwent this operation at Marie-Lannelongue Hospital in Paris. RESULTS: There was one early death and no late deaths. At a mean follow-up of 5.2 years, all survivors are in New York Heart Association class I. Early atrial flutter, related to atrial scarring secondary to multiple previous surgical procedures, developed in 1 patient, and late atrial flutter developed in 1 patient who had a previous Blalock-Hanlon atrial septectomy. All patients are currently in sinus rhythm. Atrial flutter did not occur in 17 patients who had had no previous atrial wall surgical procedure. CONCLUSIONS: We believe that the good long-term clinical results are directly attributable to our modifications, which ensure optimal hemodynamics and absence of rhythm disturbances. All patients who had not previously undergone operation on the atrial wall were free from supraventricular tachyarrhythmias at a mean follow-up of 5.2 years. This is a consequence of protecting the sinus node, crista terminalis, and Bachmann's bundle.

Transaortic closure of residual intramural ventricular septal defect.

BACKGROUND: Residual intramural ventricular septal defect is an unusual cause of left-to-right shunt after biventricular repair of conotruncal anomalies. It results from the insertion of the patch within the trabeculated right ventricular free wall related to the ventriculoinfundibular fold creating a communication through the intertrabeculated spaces to the right ventricular cavity. This complication often leads to unsuccessful reoperations unless the exact mechanism of the shunt has been identified. METHODS AND RESULTS: Five patients presented with residual intramural ventricular septal defects. Three had double outlet right ventricle, one pulmonary atresia with ventricular septal defect, and one tetralogy of Fallot. One patient was unsuccessfully reoperated on for closure of the residual ventricular septal defect through the right ventricular approach. The surgical treatment, which consisted of patch closure of the residual intramural ventricular septal defect through aortotomy, was successful in 3 patients. In the 2 remaining patients the hemodynamically insignificant residual intramural ventricular septal defect remained untouched. No mortality or morbidity occurred. CONCLUSIONS: Residual intramural ventricular septal defect should be suspected in presence of a residual ventricular septal defect after biventricular repair of conotruncal anomalies. It is not accessible through either atriotomy or right ventriculotomy. The transaortic approach allows an easy treatment of this rare complication.

Pulmonary circulation evaluation before cavopulmonary connections: the cavopulmonary bypass.

The outcome of Fontan-type procedures is dependent on several risk factors, among which pulmonary vascular resistances (PVRs) are an important component. Preoperative calculation of PVR entails several potential sources of error, particularly in patients with pulmonary atresia or multiple sources of pulmonary blood flow. In an attempt to develop a reliable test that accurately assesses the hemodynamic patterns of the pulmonary vascular bed before a Fontan procedure, a simulation of Fontan-type circulation was achieved in 13 patients by a partial cardiopulmonary bypass between the main pulmonary artery and both venae cavae (cavopulmonary bypass). During cavopulmonary bypass, pressures and resistances were recorded. Immediately after cavopulmonary bypass, the circulation was converted to standard cardiopulmonary bypass and the cavopulmonary connection was carried out. Preoperative pulmonary vascular resistance indexes were assessed roughly by the arteriovenous oxygen difference in systemic and pulmonary beds. There was no correlation between preoperative and perioperative calculations of pulmonary vascular resistance indexes (r = 0.24; p = not significant). Hemodynamic data available for all patients then were correlated to the early postoperative outcome assessed by a subjective four-point scale. A positive, significant correlation was found with intraoperative PVR (r = 0.90; p < 0.001), indexed PVR (r = 0.90; p < 0.001), and the pulmonary to systemic vascular resistance ratio (r = 0.98; p < 0.0001). Two of 13 patients had a 4-mm fenestration in the atrial baffle. No mortality or morbidity was related to the procedure. The absolute values of PVR and pulmonary vascular resistance indexes were strikingly higher than generally admitted for this type of procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Absent pulmonary valve syndrome: surgical treatment and considerations.

BACKGROUND: The operative management of absent pulmonary valve syndrome remains controversial regarding the need for pulmonary valve implantation and remodeling of pulmonary arteries. Moreover, symptomatic infants are considered to have a poor prognosis. This retrospective report summarizes the experience of a single institution. METHODS: Between May 1977 and May 1995, 37 consecutive patients underwent repair of absent pulmonary valve syndrome. Patients were divided into two groups according to age at operation: group A (10 infants less than 1 year old) and group B (27 patients older than 1 year). Mean age at operation was 5 +/- 4 months in group A and 72 +/- 42 months in group B. Initially, repair consisted of ventricular septal defect closure and relief of right ventricular outflow tract obstruction combined with pulmonary valve implantation. More recently, the concept of treatment has evolved with pulmonary arterioplasty without pulmonary valve insertion, except in patients with elevated pulmonary artery pressure. RESULTS: Of the 37 patients, 34 had successful repair. The overall in-hospital mortality rate was 8% (two deaths in group A and one in group B). No hemodynamic data were correlated with operative death. Death was associated with longer extracorporeal circulation time (p = 0.005) and longer aortic cross-clamping time (p = 0.019). In fact, these were clearly related to more complex anatomy (p = 0.001): multiple ventricular septal defects in 1, left pulmonary artery arising from the ductus in another, and left pulmonary artery arising from the aorta in the remainder. Follow-up was available in 22 of the 34 survivors. Mean follow-up time was 30 +/- 47 months in group A and 38 +/- 33 months in group B. All but 1 had no restriction of exercise, and most of them had pulmonary incompetence of Doppler echocardiography. One developed severe exercise intolerance because of pulmonary valve stenosis (xenograft), leading to uneventful reoperation 123 months after initial repair. One infant died suddenly of complete atrioventricular block 3 months after repair. The late mortality rate was 5%. CONCLUSIONS: Surgical treatment of absent pulmonary valve syndrome should include pulmonary arterioplasty to reduce bronchial obstruction, with no need for pulmonary valve insertion. This procedure is feasible and is recommended especially in markedly symptomatic infants.

Successful treatment of chaotic atrial tachycardia with oral flecainide.

Two infants with chaotic atrial tachycardia diagnosed at birth and at 11 weeks of life, were treated successfully with oral flecainide. Both patients were in heart failure at initiation of therapy. Six months and 17 months later, respectively, they have had no recurrence of chaotic atrial tachycardia, left ventricular function returned to normal and therapy was stopped in both.

Double-outlet right ventricle with non-committed ventricular septal defect.

OBJECTIVE: The term non-committed was used to define hearts in which the VSD was anatomically related to, or was close to, neither great vessel, being separated from both by considerable muscle. We report our experience of the surgical management of this subset, considered being of particular surgical relevance. METHODS: Between January 1987 and December 1997, 23 patients having double-outlet right ventricle (DORV) with non-committed VSD underwent biventricular repair. Nine (39%) had undergone previous palliation. The median age was 20 months and the median weight was 8.5 kg. Two main types of repair were used: intraventricular baffle repair (n = 21) and arterial switch operation with VSD to pulmonary artery baffle (n = 2). At repair, 12 (52%) patients required concomitant VSD enlargement. In two other patients presenting with restrictive inlet VSD associated with tricuspid attachments, crossing the subaortic pathway biventricular repair was abandoned at operation. RESULTS: There were two hospital deaths (9%, 70% CL: 3-19%). Eight patients (35%, 70% CL: 23-48%) underwent nine reoperations, six for subaortic stenosis. No late death occurred. At last visit, all patients were asymptomatic and only two had cardiac medication. CONCLUSIONS: The biventricular repair of DORV with non-committed VSD is feasible in the vast majority of cases with comparable results to other subsets of DORV. After repair, the subaortic region is at risk for development of subaortic stenosis.

Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch.

OBJECTIVES: Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta. This technique can be limited by the presence of tricuspid chordae and by the pulmonary artery to tricuspid valve distance. Furthermore, there is an important risk of late subaortic obstruction related to the long patch required that creates a potential akinetic septal area. Presented here is another technique; by VSD rerouting to the pulmonary infundibulum and arterial switch. METHODS: Ten patients, with DORVncVSD, underwent a VSD rerouting to the pulmonary infundibulum followed by arterial switch. Seven had a previous pulmonary artery banding and one a moderate infundibular stenosis. The median age at surgery was 16 months (range 3 weeks to 4.5 years). All patients had a bilateral infundibulum, with a large persistent subaortic conus, D malposition of the aorta, side-by-side vessels and double loop coronary patterns. The VSD was perimembranous with inlet or trabecular extension. Subaortic obstruction was constant. The VSD was severely distant from both the aortic and the pulmonary annulus. The operation was conducted through a combined approach. The VSD was constantly enlarged superiorly. The almost permanent subaortic obstruction was released. The VSD was always found quite close to the pulmonary infundibular ostium. The arterial switch technique was adapted to the complex coronary anatomy. RESULTS: There was one non-cardiac death. At a mean follow-up of 20 months, all nine survivors are in NYHA class I, in sinus rhythm, and have no subaortic gradient greater than 15 mm. CONCLUSION: This technique of VSD rerouting to the pulmonary artery and arterial switch limits greatly the size of the rerouting patch, respects the tricuspid chordae and is independent of the pulmonary artery-tricuspid valve distance. In this early series of biventricular repair of DORVncVSD, the VSDs were always found close to the pulmonary artery, allowing this new type of repair.

Use of transtelephonic electrocardiographic monitoring in children with suspected arrhythmias.

Arrhythmias in children can produce various symptoms and their assessment often is difficult with usual methods. Transtelephonic electrocardiographic monitoring was used in 136 symptomatic patients (palpitations [112], syncope [six], chest pain [18]) for a mean duration of 86 +/- 65 days; mean age was 11.1 +/- 4.8 years and 62% were female. Of 398 recordings sent (mean 2.9 per patient) 32 revealed significant arrhythmias (8%). Of the 67 patients reporting symptoms during the recording period, only 24 had documented arrhythmias - 23 supraventricular tachycardia and one junctional tachycardia. The negative predictive value is 100%, compared with a low positive predictive value of 36%; sensitivity and specificity are, respectively, 100 and 62%. Of the 24 patients with documented arrhythmias, 54% were treated versus 13% of those with normal recordings (P < 0.01). At the end of the follow-up (mean duration 1.6 +/- 1.2 years), 63% of the patients with negative recordings had not complained of further symptoms versus 22% of those with documented arrhythmias (P = 0.001). Transtelephonic electrocardiographic monitoring is an important tool for documenting infrequent arrhythmias in children and even more for reassuring the patient and his or her family in the absence of significant arrhythmias.