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Background In a normal neonatal heart the ductus arteriosus forms the direct continuity of the pulmonary trunk to the descending aorta being the main channel for the prenatal blood flow. The pulmonary arteries originate separately from the pulmonary trunk. In congenital heart malformations with pulmonary stenosis to atresia there is often an abnormal lateral DA to left pulmonary artery connection combined with a localised narrowing (pulmonary ductal coarctation / PDC) or even interruption. We investigated embryonic remodelling and pathogenesis of this area Material and methods Normal development was studied in WntCre reporter mice (E10.0-12.5) for neural crest cells and Nkx2.5 immunostaining for second heart field cells. Data were compared to stage matched human embryos including histopathology of neonatal specimen, and a VEGF120/120 mutant mouse strain developing pulmonary atresia. Results Normal mouse and human embryos showed that, in the early embryo, the mid-pharyngeal endothelial plexus, partly covered by second heart field, connected side-ways to the 6 th pharyngeal arch artery (PAA). The ventral segment of this PAA effectively formed the proximal pulmonary artery. The origin of the cells encasing the endothelial tubes differed significantly. The dorsal 6 th PAA segment (future DA on the left side) was solely surrounded by neural crest cells. The ventral segment had a dual outer lining with neural crest cells laterally and second heart field cells medially, while the distal pulmonary artery was covered by neither of these cells. The asymmetric contribution of second heart field to the future pulmonary trunk on the left side of the aortic sac (so-called pulmonary push) was evident. During remodelling the ventral segment of the 6 th PAA became incorporated into the pulmonary trunk leading to a separate connection of the left and right pulmonary arteries to the latter. The VEGF120/120 embryos showed a severely stunted pulmonary push and later on a variety of vascular anomalies in the DA to pulmonary artery connections. Conclusion Side-way connection of the DA to the left pulmonary artery is a congenital anomaly. The primary problem is a stunted development of the second heart field-derived pulmonary push leading to pulmonary stenosis/atresia and a subsequent lack of proper incorporation of the ventral segment of the 6 th PAA into the aortic sac. Clinically, the aberrant smooth muscle tissue of the DA should be addressed to prohibit development of severe PDC or even interruption of the left pulmonary artery.

Objective To determine the prevalence of congenital heart defects (CHDs) in patients with mild or severe congenital anorectal malformations (CARMs), and whether all patients with CARM need pediatric cardiology screening.Study design We included 129 patients with CARM born between 2004 and 2013, and referred to University Medical Center Groningen. Recto-perineal and recto-vestibular fistulas were classified as mild CARMs, all others as severe. Significant patent foramen ovale, secundum atrial septal defect, and small ventricular septum defect were classified as minor CHDs, all others as major.Results Of 129 patients with CARM, 67% had mild CARM, 33% severe CARM, and 17% were additionally diagnosed with CHD. CHDs were distributed equally in patients with mild or severe CARMs. Patients with multiple congenital abnormalities were more frequently diagnosed with CHD (n = 16, 36%) than patients without multiple congenital malformations (n = 5, 9%, P = .001). Patients with CARM diagnosed with CHD using pediatric cardiac echo screening were younger than 3 months of age at diagnosis. Earlier general pediatric examinations missed 7 (50%) children with mild and 4 (50%) with severe CHDs.Conclusions The severity of CARM could predict neither prevalence nor severity of CHD. More than one-half of CHDs were missed during the first physical examination. No new CHDs were found in patients older than 3 months of age at the time CARMs were diagnosed. We recommend screening all patients with CARM younger than 3 months of age for CHD at the time CARM is diagnosed. Preoperative echocardiography should be the rule in children younger than 3 months of age and with multiple congenital anomalies.

Background— The value of echocardiography in assessing disease severity and predicting outcome in pediatric pulmonary arterial hypertension (PAH) is insufficiently defined. The aim of this study was to describe correlations between echocardiography and disease severity and outcome in pediatric PAH. Methods and Results— Forty-three consecutive children (median age, 8.0 years; range, 0.4–21.5) with idiopathic/hereditary PAH (n=25) or PAH associated with congenital heart disease (n=18) were enrolled in a prospective single-center observational study. Anatomic and right ventricular-functional variables were obtained by two-dimensional echocardiography and Doppler-echocardiography at presentation and at standardized follow-up and were correlated with measures of disease severity (World Health Organization functional class [WHO-FC], N-terminal-pro-B-type natriuretic peptide, hemodynamics) and lung-transplantation–free survival. Right atrial and right ventricular dimensions correlated with WHO-FC and hemodynamics ( P P P P P =0.071). Tricuspid annular plane systolic excursion correlated with WHO-FC, mean right atrial pressure and survival ( P Conclusions— This early descriptive study shows that echocardiographic chararacteristics of both the right and the left heart correlate with disease severity and outcome in pediatric PAH, both at presentation and during the course of the disease. The preliminary data from this study support the potential value of echocardiography as a tool in guiding management in children with PAH.

Background. Functional univentricular hearts are currently palliated by a staged procedure of which the bidirectional cavopulmonary shunt is usually the second stage. In addition to this stage, a calibrated amount of additional pulmonary blood flow may be preserved to promote pulmonary artery growth and increase the length of the interval preceding the total cavopulmonary connection. However, additional pulmonary blood flow can be deleterious for ventricular functioning and development as it increases functional ventricular volume load.Methods. Using the Fick principle we devised a theoretic framework to estimate the ventricular volume loading caused by additional pulmonary and collateral aortopulmonary flow. To use this framework, blood samples need to be taken intraoperatively from the aorta, pulmonary veins, and inferior caval vein to determine oxygen saturations. The oxygen saturation samples have to be taken sequentially with and without additional pulmonary blood flow.Results. The objective of this paper is to provide a theoretic framework to estimate the ventricular volume loading caused by collateral aortopulmonary flow and additional pulmonary blood flow in the context of a bidirectional cavopulmonary shunt in the staged palliation of univentricular hemodynamics. The formulas have not yet systematically been applied in vivo.Conclusions. The added volume loading of the ventricle caused by additional pulmonary blood flow can theoretically be estimated using the newly devised formulas so as to calibrate ventricular volume loading to a desired level intraoperatively. (Ann Thorac Surg 2012;93:2022-7) (C) 2012 by The Society of Thoracic Surgeons

Aims Right ventricular (RV) dysfunction is a major determinant of long-term morbidity and mortality in congenital heart disease. The right ventricle (RV) is genetically different from the left ventricle (LV), but it is unknown as to whether this has consequences for the cellular responses to abnormal loading conditions. In the LV, calcineurin-activation is a major determinant of pathological hypertrophy and an important target for therapeutic strategies. We studied the functional and molecular adaptation of the RV in mouse models of pressure and volume load, focusing on calcineurin-activation. Methods and results Mice were subjected to pulmonary artery banding (PAB), aorto-caval shunt (Shunt), or sham surgery (Control). Four weeks later, mice were functionally evaluated with cardiac magnetic resonance imaging, pressure measurements, and voluntary cage wheel exercise. Right ventricular hypertrophy and calcineurin-activation were assessed after sacrifice. Mice with increased pressure load (PAB) or volume load (Shunt) of the RV developed similar degrees of hypertrophy, yet revealed different functional and molecular adaptation. Pulmonary artery banding increased expression of Modulatory-Calcineurin-Interacting-Protein 1 (MCIP1), indicating calcineurin-activation, and the ratio of beta/alpha-Myosin Heavy Chain (MHC). In addition, PAB reduced exercise capacity and induced moderate RV dilatation with normal RV output at rest. In contrast, Shunt did not increase MCIP1 expression, and only moderately increased beta/alpha-MHC ratio. Shunt did not affect exercise capacity, but increased RV volumes and output at rest. Conclusions Pressure and volume load induced different functional and molecular adaptations in the RV. These results may have important consequences for therapeutic strategies to prevent RV failure in the growing population of adults with congenital heart disease.

We report a 29 week-gestation preterm infant who presented during his second week of life with cardiogenic shock. Clinical presentation and first diagnostics suggested myocardial infarction, but echocardiographic features during follow-up pointed to a diagnosis of enteroviral myocarditis. The child died of chronic heart failure at 9 months of age. Autopsy showed passed myocardial infarction. No signs for active myocarditis were found. We discuss the difficulties in differentiating between neonatal myocardial infarction and myocarditis. Recognizing enteroviral myocarditis as cause for cardiogenic shock is of importance because of the therapeutic options.

Objective: Total cavopulmonary connection (TCPC) is a routine operation for palliation of children with cardiac anomalies in whom biventricular repair is impossible. The original technique consists of the creation of a semi-prosthetic intercaval tunnel. A substantial proportion of these patients develop atrial flutter. We developed a technique for creating an intercaval tunnel that uses the tissue of the right auricle as intercaval tunnel. This technique avoids suture lines in the neighbourhood of the blood supply of the sinus node and leaves the terminal crest free. Since atrial flutter frequently occurs after Mustard and Senning operations in which suture lines are similar as for creating the lateral tunnel in TCPC we postulated that our technique for coating the intercaval tunnel without prosthetic material might prevent atrial flutter. Methods: All the children that qualified for a TCPC were included. Whenever possible our operative technique was applied. In the other cases a semi-prosthetic conduit was used for creating the intercaval tunnel. Of 47 consecutive patients 30 (64%) had a tunnel of right auricle tissue, 12 (26%) had a tunnel of prosthetic material. Five patients did not need an intercaval tunnel and were omitted in this study. Only surviving patients were included in this study. Patients that needed more atrial surgery then necessary for TCPC were also omitted. Postoperative ECG's and Holter monitorings were studied. Results: Overall mortality was 7 of 47 patients (14.9% 70% CL 9.4-22.2%). There was no mortality due to rhythm disturbances. Atrial flutter occurred in 3 of 31 included patients (9.7, 70% CL 4.3-18.5%). In the right auricle group 1 of 22 patients (4.5, 70% CL 0.6-14.6%) had atrial flutter compared to 2 of 9 patients (22,2, 70% CL 7.5-45.0%) in the prosthesis group (P = 0.13). Conclusion: The use of the right auricular technique for creating the intercaval tunnel TCPC is applicable in the majority of patients qualifying for a TCPC, Mortality and morbidity are equal comparing the two techniques. However, markedly less atrial flutter occurs in the group where the right auricle was used as intercaval tunnel. Therefore, we recommend the use of our technique for total cavopulmonary connection. (C) 1998 Elsevier Science B.V. All rights reserved.

Coarctation of the aorta and associated ventricular septal defect may be repaired simultaneously or by initial coarctation repair with or without banding of the pulmonary artery. The question is whether specific preoperative criteria can enable the surgeon to choose the optimal surgical management. Between 1980 and 1993, 80 infants younger than 3 months with coarctation and ventricular septal defect were treated surgically. In 64 infants (multistage group), simple coarctation repair was performed through a posterolateral approach, with concomitant banding of the pulmonary artery in 10 infants. Twenty ventricular septal defects were closed as a secondary procedure and four were closed as a tertiary procedure. Sixteen infants (single-stage group) underwent one-stage repair through an anterior midline approach. The total in-hospital mortality rate was 7.5%. Freedom from recoarctation after 5 years was 91.3% in the multistage group versus 60.0% in the single-stage group (p = 0.018). Freedom from secondary ventricular septal defect treatment in the multistage group after 5 years was 40.7%, versus 100% in the single-stage group (p = 0.016). Thirty-seven ventricular septal defects (47.8%) closed spontaneously. In particular, the preoperative left-to-right shunt and extension of the perimembranous VSD into the inlet or outlet were risk factors for the need for eventual surgical ventricular septal defect closure after initial coarctation repair. On the basis of these two risk factors, the probability of the need for eventual surgical treatment of ventricular septal defect after initial coarctation repair can be calculated. This policy offers a well-considered choice between single-stage and multistage repair, weighing the risk of secondary ventricular septal defect treatment versus the risk of recoarctation. Finally, the number of surgical procedures per infant will be as low as possible. (J THORAC CARDIOVASC SURG 1996;111:168-75)

Now that systemic-pulmonary collateral arteries are used for unifocalization in patients with pulmonary atresia and ventricular septal defect, the question arises whether morphologic criteria of these collateral arteries could help to provide better results. In an attempt to classify the morphologic features of systemic-pulmonary collateral arteries, we studied 31 heart-lung autopsy specimens with pulmonary atresia and ventricular septal defect. The course of the systemic-pulmonary collateral arteries (origin, branching pattern, and connections with systemic and central pulmonary arteries) was related to their histologic characteristics. The results show that systemic-pulmonary collateral arteries cannot be classified according to their course related to the trachea and the main branches of the bronchi. The histologic features of these collateral arteries vary along their course to the lungs. Nearly all systemic-pulmonary collateral arteries contain a muscular or a musculoelastic segment. One type of collateral artery (complex loop anastomoses) is completely muscular and resembles a bronchial artery. Nutritive branches (bronchial arteries) arise from all histologic types of systemic-pulmonary collateral artery segments. The size and number of intimal proliferations in muscular, elastic, and musculoelastic segments did not differ significantly. In 29 of 31 cases a ductus arteriosus did not coexist with large collateral arteries (two cases unknown). It is concluded that a classification of large systemic-pulmonary collateral arteries based on morphologic features results in a highly variable system, which does not facilitate decisions for the suitability of these arteries for unifocalization procedures. The variability of the systemic-pulmonary collateral arteries corresponds with the recent embryologic finding that during development, collateral artery formation is possible during extended periods.

Background. The staged Fontan procedure is used to palliate functionally univentricular hearts. The effect of additional pulmonary blood flow combined with a bidirectional cavopulmonary shunt in these patients remains a controversial subject.Methods. This retrospective study included all 82 patients with a unilateral or bilateral bidirectional cavopulmonary shunt at our institution between April 1990 and July 2010. Patients with hypoplastic left heart syndrome were excluded. Two groups, based on the presence (n = 57) or absence (n = 25) of additional pulmonary blood flow after the bidirectional cavopulmonary shunt, were compared.Results. Patients with a bidirectional cavopulmonary shunt combined with additional pulmonary blood flow had higher arterial oxygen saturations postoperatively (86% [interquartile range, 85% to 90%] vs 82% [80% to 85%]; p = 0.001) and had a longer median interval before the total cavopulmonary connection (3.42 [2.43 to 4.89] years vs 2.90 [2.08 to 3.32] years; p = 0.06). At the total cavopulmonary connection, they were older (4.59 [3.88 to 6.49] years vs 3.94 [3.10 to 4.57] years; p = 0.03) and had a larger median body surface area (0.73 [0.65 to 0.87] m(2) vs 0.68 [0.59 to 0.73] m(2); p = 0.04).Conclusions. Patients with a bidirectional cavopulmonary shunt and additional pulmonary blood flow have a longer interval before the total cavopulmonary connection without evident untoward effects. This may theoretically be advantageous for the pulmonary artery growth needed for a successful Fontan circulation. Furthermore, postponement of the final Fontan may ensure the insertion of a larger extracardiac conduit to avoid prosthesis-patient mismatch. (Ann Thorac Surg 2012;93:2028-34) (C) 2012 by The Society of Thoracic Surgeons

With the use of dedicated pediatric single-plane probes, transesophageal echocardiography was performed prospectively in 102 children to determine the potential value of this new technique in the diagnosis (40 patients) and the follow-up of congenital heart disease (29 patients) and as a monitoring technique during the perioperative period or interventional cardiac catheterization (33 patients). The findings were correlated with precordial studies (102 patients), cardiac catheterization (82 patients), epicardial ultrasound findings (18 patients), or surgical inspection (34 patients). The age at investigation ranged from 2.5 months to 14.9 years (mean age, 5.2 years); weight ranged from 3.7 to 52 kg (mean weight, 19.1 kg). Additional information was obtained in 49 patients (48.4%) and was relevant for patient management in 21 patients (20.6%). Major areas of improved diagnostic insight included the following: (1) systemic and pulmonary venous return, (2) atrial morphology, (3) atrioventricular junction abnormalities, (4) left ventricular outflow tract disease, (5) atrial baffle function, and (6) the Fontan circulation. Inherent limitations were as follows: (1) the semiinvasive character of the procedure, (2) the need for heavy sedation or general anesthesia, and (3) the limited imaging planes provided. Transesophageal echocardiography in children can provide important additional diagnostic information on a wide spectrum of congenital heart lesions. The technique would appear to be a highly valuable adjunct to the diagnostic armentarium of the pediatric cardiologist.

Previous methods used to assess atrial baffle function after correction of transposition of the great arteries have included precordial echocardiography and cardiac catheterization. To evaluate whether single plane transesophageal echocardiography might provide additional information, its findings were correlated with information derived from both precordial echocardiography and cardiac catheterization in 15 patients (14 Mustard procedures, 1 Senning procedure) aged 4.2 to 33 years (mean 16.3). Precordial ultrasound with combined imaging, color flow mapping and pulsed Doppler ultrasound visualized the supramitral portion of the common systemic venous atrium in every case but could identify only superior limb obstruction in three of six patients, mid-baffle obstruction in zero of two and inferior limb obstruction in zero of two patients. Transesophageal studies with use of the same range of ultrasound methods demonstrated superior limb obstruction (severe in four, mild in two) in six of six patients, mid-baffle obstruction in two of two and inferior limb obstruction in two of two patients. The entire pulmonary venous atrium was equally well interrogated by either ultrasound approach, with both identifying three cases (two mild, one moderate) of mid-pulmonary venous atrium obstruction. However, individual pulmonary vein velocity profiles could only be recorded by transesophageal pulsed Doppler ultrasound. Precordial studies identified baffle leaks (1 large, 2 small) in only three patients, whereas transesophageal studies identified 11 such baffle leaks (1 large, 10 small), which were multiple in two patients. It is concluded that transesophageal echocardiography provides a more detailed and accurate assessment of atrial baffle morphology and function than is provided by either precordial ultrasound or cardiac catheterization. Transesophageal echocardiography appears to be the investigative method that derives the most information and that is applicable on an outpatient basis in adolescent and adult patients.

Transesophageal echocardiography with a single plane (transverse axis), dedicated pediatric probe was performed prospectively in 25 anesthetized children undergoing routine cardiac catheterization or intracardiac surgery, to assess the potential role of this technique in the initial diagnosis, perioperative management and postoperative follow-up of children with congenital heart disease. The group ranged in age from 1 year to 14.8 years (mean 6.1) and weight from 6.5 to 52 kg (mean 22.4). Studies were successful in all patients and no complications were encountered. The results of the transesophageal studies (combined imaging, color flow mapping and pulsed wave Doppler sampling) were correlated both with the results of prior precordial studies and the information obtained at cardiac catheterizatton. Transesophageal echocardiography provided a more detailed evaluation of the morphology and function of systemic and pulmonary venous return, the atria, interatria) baffles, atrioventricular valves and the left ventricular outflow tract. Additional information was obtained in 15 patients (60%). Problem areas for single plane transesophageal imaging were the apical interventricular septum, the right ventricular outflow tract and the left pulmonary artery. The intraoperative use of transesophageal echocardiography allowed assessment of the surgical repair and monitoring of ventricular function and volume status while the patient was weaned from cardiopulmonary bypass. Transesophageal echocardiography in pediatric patients is of additional value in three main areas: 1) toe precise morphologic diagnosis of congenital heart disease, 2) perioperative monitoring, and 3) postsurgical follow-up.

J Thorac Cardiovasc Surg 1997;113:417-9

The effectiveness of continuous indomethacin (INDO) infusion versus bolus infusions for closure of patent ductus arteriosus (PDA) was investigated. The study design was an open-label case series (continuous INDO) with historic controls matched for gestational age (bolus INDO). Ductal closure rates were determined in two groups: 16 preterm infants with PDA treated with continuous INDO infusion (CONTIN group) and 16 control patients, matched for gestational age, who received bolus INDO infusions (BOLUS group). The total dosage was the same for both groups. PDA closed in seven of 16 preterm infants in the CONTIN group and in 13 of 16 in the BOLUS group (p = 0.033, Fisher's exact test). In infants

Closure of so-called “secundum” atrial septal defects with a device inserted on a catheter necessitates precise delineation of their morphology. Echocardiography is the diagnostic method of choice to demonstrate this morphology, and to differentiate such defects located within the oval fossa from the other variants producing an interatrial communication. Precordial echocardiography usually allows selection of cases likely to be suitable for closure in this fashion. This selection is based on the localisation and the size of the deficiency in the oval fossa, the length of the interatrial septum, and the adequacy of the infolded rims surrounding the defect. Suitability for closure is reevaluated by transesophageal echocardiography, either asa separate investigation or at the start of the interventional theterisation. This investigation requires a multiplane transesophageal echocardiographic probe, since only oblique planes will demonstrate the entrance of the systemic and pulmonary veins and their relationship to the defect. Transesophageal echocardiography serves as an important monitoring tool during the interventional procedure. As such, it is a necessary adjunct to fluoroscopy. The stretched diameter of the defect measured with a balloon is the main eterminant of the choice of the type and size of the device. This diameter can be measured fluoroscopically, as well as on echo. Colorflow mapping serves to rule out residual shunting during the occlusion of the defect with the balloon. During deployment of the device, constant echocardiographic visualisation of the device and its position relative to the atrial septum facilitates proper placement. Such constant visualisation can only be provided by repeated quick acquisitions of multiple planes. Once the device is released, the investigator should continue to record the position of the device, and assess the potential for residual shunting.Most of the devices show some subtle change in position during the first 20 minutes after implantation.

We used transoesophageal echocardiography in 4 children with either documented or suspected obstruction of the left ventricular outflow tract to determine whether this technique may provide additional morphologic information when compared to praecordial ultrasound. Transoesophageal studies defined unsuspected mitral valvar abnormalities in two patients and involvement of the subvalvar mitral apparatus in one. In one patient, the study documented a suspected fibrous ridge associated with malalignment of the outlet septum. Transoesophageal echocardiography would appear to be a new technique with definite advantages in defining the spectrum of lesions which cause obstruction to the left ventricular outflow tract in children.

Forty-one specimens with pulmonary atresia and confluent pulmonary arteries were studied to establish the frequency and the nature of anomalies of both the ductus arteriosus and the central pulmonary arteries and their possible interrelation. The specimens were divided according to the type of pulmonary atresia: valvar atresia (12 cases) versus "muscular" atresia of the pulmonary orifice (29 cases). Abnormal connexions between the duct and the adjacent great arteries, as well as stenosis of a pulmonary artery, were common in cases with atresia of the pulmonary orifice and relatively rare in cases with imperforate valve. The abnormal connexions consisted of a proximal displacement of the origin of the duct from the aorta and displacement of the entry site of the duct into the pulmonary trunk towards one of the main pulmonary arteries. A left duct thus connected to the left and a right duct to the right pulmonary artery. Pulmonary artery stenosis, exclusively occurring in those specimens with a duct connecting to a pulmonary artery, were located in the segment of pulmonary artery between the pulmonary trunk and the duct. Histological examination revealed ductal tissue in the wall of a pulmonary artery in more than half of the cases with atresia of the pulmonary orifice. Ductal tissue in a pulmonary artery and pulmonary artery stenosis frequently coexisted. It is concluded that stenoses in the central pulmonary arteries may be related to the duct. The relationship resembles that between the duct and coarctation of the aorta.

Localised coarctation of the aorta was studied histologically in 45 cases. The age range of the patients, from 2 weeks to 40 years, enabled us to make a comparison between early and late findings. The basic histological architecture of all coarctations was identical. The ridge consisted mainly of thickening plus some infolding of the aortic wall. Ductal tissue formed the inner part of the ridge for more than half of its total circumference. In older cases masses of secondary intimal proliferation were present, which narrowed the residual lumen of the coarctation. The coarctation was located preductally in most young patients and postductally in the majority of older patients. It is concluded that ductal tissue is invariably present in the ridge and that all coarctations develop in a preductal position. Secondary changes include a gradual shift of the ridge from a preductal to a postductal position, and progressive narrowing of the residual lumen by intimal proliferation.

Intraoperative two-dimensional contrast echocardiography was used to detect a residual shunt in 50 patients after surgical repair of ventricular septal defect. Contrast injections were performed following termination of the extracorporeal circulation. In the presence of a shunt the intensity of opacification of both left and right ventricular cavities was compared. In 40 patients no ventricular shunting was observed; insignificant shunting was noted in five patients. Follow-up of these 45 patients proved uneventful. Significant opacification of the right ventricle was noted in five patients. This finding, however, does not necessarily indicate a residual shunt of significant volume. In two patients the residual shunt was confirmed postoperatively by pulsed Doppler echocardiography but clinically there was no need for surgery. Three other patients subsequently required reoperation and partial patch dehiscence was confirmed in all. Thus, intraoperative two-dimensional contrast echocardiography is a sensitive technique to detect a residual ventricular septal defect, an observation which may warrant reoperation before chest closure.

The case is described of a boy with some right ventricular dysplasia and episodes of ventricular tachycardia of left bundle branch block pattern who had symptoms from the age of 1 month. Angiography and cardiac biopsy demonstrated major involvement of the left ventricular myocardium. A sister of the patient presented at the age of 2 months with predominantly left ventricular cardiomyopathy; clinical signs and angiography suggested the presence of right ventricular dysplasia as well. She died suddenly at the age of 9 years. Her brother, now aged 14 years, is being treated with antiarrhythmic drugs. The hypothesis of this cardiomyopathy being a variant of "arrhythmogenic right ventricular dysplasia syndrome" is discussed.

Forty-three specimens with an obstructive aortic arch anomaly, obtained from infants that had died under the age of one year, were studied to establish the relationship between the arch anomalies and the ductus arteriosus. All specimens were studied morphologically. Twenty of the 43 were also studied histologically. The morphological part of the study included measurements of all the segments of the aortic arch and establishment of the localisation of any associated coarctation. The specimens were divided according to the appearance of the isthmus. The isthmus was not present in 4, was atretic in 2, hypoplastic in 19 and had a normal diameter in 18 cases. In all cases, except 5 (2 with atresia and 3 with hypoplasia of the isthmus) there was an associated coarctation. The majority of the coarctations (28) was located preductally. All such preductal coarctations had ductal tissue in the obstructing ridge. There were 5 paraductal coarctations. Of these, two also had ductal tissue in the ridge. The distribution of the ductal tissue among the two types of coarctation suggested a spectrum of anomalies rather than two completely different entities. The patterns of the aortic arches appeared predictive for the type of coarctation present. The arches that tapered normally towards the isthmus either presented with a preductal coarctation (20 cases) or without a coarctation (3 cases). All 4 specimens in which the isthmus was lacking also had a preductal coarctation. The 5 paraductal coarctations were found in arches that showed a "reverse" pattern, tapering towards the brachiocephalic arteries. All the preductal coarctations contained ductal tissue whereas only 2 of the 5 paraductal coarctations had ductal tissue in the obstructing ridge. It is concluded that careful evaluation of all the segments of the aortic arch may be helpful in the clinical evaluation of infants with an obstructive aortic arch anomaly.

The aortic arches of 34 specimens with hypoplastic left heart syndrome were studied in order to establish the frequency, the nature and the clinical implications of aortic arch anomalies. A localized aortic coarctation was present in 23 specimens. The coarcation was located preductally in 2 and paraductally in 21 cases. The degree of obstruction caused by the coarctation varied considerably. In only 6 cases (1 preductal and 5 paraductal) was the obstruction judged to be severe. One of these cases had an additional aneurysm of the aortic wall proximal to the coarctation. An aberrant relation of the ductus arteriosus and the aortic arch was found in 2 specimens without localized coarctation. In the remaining 9 specimens the aortic arch appeared normal. The aortic arch anomalies were mostly present in specimens with a severely hypoplastic ascending aorta. Clinicians, when preparing infants with hypoplastic left heart syndrome for surgical palliation, should always suspect associated aortic arch anomalies, especially when there is severe hypoplasia of the ascending aorta. Coarctation of the aorta will require additional surgical treatment.