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Knowledge of normal aortic dimensions is important in the management of children with aortic disease. So as to define such dimensions, we undertook a retrospective review of clinical data and aortic cineangiograms from 167 subjects without aortic disease having a mean age of 3.67 years, with a range from 0.01 to 14.95 years. Amongst the patients, 56 were without detectable cardiac lesions, 66 patients had mild pulmonary stenosis, 30 were seen with Kawasaki disease, and 15 with small interatrial defects within the oval fossa. Aorto-grams were available in all. No patient had any hemodynamic derangement that would have affected the aorta during intrauterine life or childhood. Systolic dimensions were measured in the ascending and descending aorta at the level of the carina, at the transverse aortic arch distal to the brachiocephalic, of the left common carotid artery at its origin, at the transverse aortic arch distal to the left common carotid artery, at the aortic isthmus, and of the aorta at the level of the diaphragm. A regression analysis model was used to establish the range of predicted normal values, with their confidence limits, standardizing the values to height as the biophysical parameter having the highest correlation to aortic dimensions. Normal ranges were established for all the levels of measurement. The data should prove useful in identifying abnormalities of the thoracic aorta during childhood, and when assessing the outcomes of interventions.
Anomalous origin of one pulmonary artery from the ascending aorta is an uncommon lesion with an uncertain outcome. We reviewed 16 consecutive children (9 males) presenting with this lesion over a 36 year period at a single institution. Median age at presentation was 2 days (range, birth to 3.2 years). The anomalous pulmonary was the right in 12 and the left in 4, all originating from the proximal ascending aorta, with no patient having stenosis at the origin of the anomalous pulmonary artery. Associated cardiac anomalies were noted in 9 patients. No intervention was attempted in 2 patients: one was deemed inoperable due to complex associated lesions and pulmonary vascular obstructive disease, while the other one died before repair. Surgical intervention was attempted in 14 patients, with 3 intraoperative deaths (21%). Of 11 operative survivors, 8 developed pulmonary arterial stenosis graded severe in 2, moderate in 1 and mild in 5. Patients with severe stenosis required surgical angioplasty, 1 after unsuccessful dilation combined with placement of an endovascular stent. One patient with moderate, and one with mild, stenosis underwent successful transcatheter balloon dilation. The remaining 4 patients with mild stenosis remain unchanged during follow-up. One patient had biopsy evidence of pulmonary vascular obstructive disease at 3.3 years of age. There were no late deaths, giving a total mortality of 25% (4/16).
While early diagnosis and repair of anomalous origin of one pulmonary artery from the ascending aorta is necessary, restenosis of the site of repair is common.
A patient with pulmonary atresia and intact ventricular septum was found to have a right ventricular-dependent coronary circulation. In this infant both coronary arteries lacked their normal proximal connection with the aorta, perhaps the most egregious form of this prejudicial coronary circulation. Even more interesting was a direct collateral vessel originating from the descending thoracic aorta and connecting with the coronary circulation. This patient has undergone bilateral modified Blalock-Taussig shunts, and left ventricular function seems preserved.
Aortopulmonary septal defect and tetralogy of Fallot is a rare combination. We report a case of tetralogy of Fallot, non-confluent pulmonary arteries with the left arising from the arterial duct, and a large aortopulmonary septal defect diagnosed by echocardiogram and confirmed by cardiac catheterization.
We report two cases with persistence of the hepatic venous complex, underdevelopment of the inferior caval vein and azygos continuation to the superior caval vein. We suggest that the hepatic venous complex is a true congenital malformation rather than the result of passive enlargement of venous channels subsequent to high venous pressures. Preoperative diagnosis is important in patients with complex congenital heart disease undergoing palliative venous redirection procedures if a postoperative right-to-left shunt and cyanosis are to be avoided.
Implantation of the Rashkind double umbrella device for closure of the persistently patent arterial duct is now routine in many units. The use of this device in non-ductal positions, however, has been reported in only a limited number of patients, and its impact on the subsequent clinical course has not been established. A retrospective review of all such occlusions, therefore, was undertaken to address the early results and impact on clinical management. From 9/1988 through 2/1994, implantation of a double umbrella device was attempted in 21 patients (eight female, 13 male) with cardiovascular communications other than a persistent arterial duct excluding the intentional fenestrations made in the modified Fontan procedure. Median age at implantation was 2.6 years (three months-12.4 years) and weight was 11.6 kg (4.1–45) kg. Lesions included four ventricular septal defects, an aortopulmonary window, an aortopulmonary collateral artery, an atrial septal defect, four Gore-tex® shunts, and a residual persistent communication between the superior caval vein and the right atrium following either a bidirectional cavopulmonary anastomosis, or a classical Glenn operation. Eight patients underwent occlusion of unintentional residual right-to-left (six) or left-to-right (two) communications following the Fontan procedure. Implantation was accomplished in all without mortality, utilizing either 12 mm (13) or 17 mm (eight) devices. Surgical removal of the device was required in one patient four weeks subsequent to implantation. At latest follow-up (1–66 months, median four months), color-flow Doppler studies revealed complete occlusion in 17 (77%) patients. Implantation of the double umbrella in sites other than the duct is, therefore, feasible and simplified technically with a modification of the delivery system. Such novel applications of transcatheter intervention using double umbrella devices may avoid or complement subsequent surgery.
We describe a multicystic tumor of the atrial septum associated with a fatal cardiac malformation. The tumor contained bronchogenic cysts and two types of vascular hamartomas. A complex form of double outlet right ventricle was present. We speculate that this tumor formed at a critical time in cardiac embryogenesis and contributed to the abnormal formation of the heart.
To determine factors associated with outcomes after listing for transplantation in children with cardiomyopathies.
Childhood cardiomyopathies form a heterogeneous group of diseases, and in many, the prognosis is poor, irrespective of the etiology. When profound heart failure develops, cardiac transplantation can be the only viable option for survival.
We included all children with cardiomyopathy listed for transplantation between 12/89 and 4/98 in this historical cohort study.
We listed 31 patients, 15 male and 16 female, 27 with dilated and 4 with restrictive cardiomyopathy, for transplantation. The median age at listing was 5.7 years, with a range from fetal life to 17.8 years. Transplantation was achieved in 23 (74%), with a median interval from listing of 54 days, and a range from zero to 11.4 years. Of the patients, 14 were transplanted within 30 days of listing. Five patients (16%) died before transplantation. Within the Canadian algorithm, one of these was in the third state, and four in the fourth state. One patient was removed from the list after 12 days, having recovered from myocarditis, and two remain waiting transplantation after intervals of 121 and 476 days, respectively. Patients who died were more likely to be female (5/5 vs. 11/26; p=0.04) and to have been in the third or fourth states at listing (5/5 vs. 15/26; p=0.04). The use of mechanical ventricular assistance, in 10 patients, was not a predictor of an adverse outcome. While not statistically significant, survival to transplantation was associated with treatment using inhibitors of angiotensin converting enzyme, less mitral regurgitation, a higher mean ejection fraction and cardiac index, and lower right ventricular systolic pressure.
Children with cardiomyopathy awaiting transplantation have a mortality of 16% related to their clinical state at the time of listing.
Dick Rowe (Fig.) would be embarrased by this attention, as he was such a very modest man. Yet it is appropriate that the late Richard Desmond Rowe be included in Cardiology in the Young's Paediatric Cardiology Hall of Fame. As I consider the life and contributions of Dick Rowe, I am at first troubled, indeed saddened, by the realization that more than a decade has passed since his untimely and premature death just weeks before his planned retirement. I have his picture on the mantle of my office. Not a day goes by that I don't look at his countenance, however fleeting, reflecting on my good fortune to have worked with and considered Dick Rowe as mentor, colleague, teacher and friend. This sense of good fortune is shared by virtually all those touched by his warmth and generosity.
The purpose of this study was to determine the efficacy of balloon expandable stents in the relief of obstructed conduits in the right heart, and to review both the immediate success and longevity of this palliation.
Methods and results
Endovascular stents where placed in 17 children, median age 4.7 yrs (range 0.46–16.2 yrs), at a median of 1.6 yrs (range 0.33–6.7 yrs) after placement of conduits at surgery. A second stent was required in two children, either because of migration of the initial stent or residual proximal stenosis. Mean right ventricular to systemic pressure ratios were reduced from 0.77 ±0.24 to 0.50±0.13, and the minimum area of the pulmonary outflow tract increased from 24±12% to 69±26% of that expected for body surface area. Further surgery was needed in 8 patients following placement of the stent, in 5 within 10 months of implantation, due to inadequate relief of obstruction. Nonetheless, 3 of these 8 children achieved satisfactory palliation 18, 21 and 24 months prior to requiring insertion of a new conduit. Actuarial freedom from reoperation was 44% at 24 months. The remaining 9 children continue to benefit from the relief in obstruction provided by the stent at a mean interval from implantation of 24±8 months. In this group, the mean Doppler gradient across the right ventricular outflow tract was 35 ±13 mmHg (n=6).
These early data indicate that endovascular stents are useful in the palliation of obstructed right ventricular conduits. In the short-term, the relief of obstruction achieved is well maintained, and has either delayed or obviated the need for insertion of new conduits.
We describe the importance of angiographic identification of collateral venous channels by balloon occlusion venography after bidirectional cavopulmonary connections. Use of the balloon occlusion technique is essential for identification of these vessels, as they can easily be missed by standard venous angiography, with possible clinical consequences for postoperative management.
Cross-sectional echocardiography has made a major contribution to the preoperative evaluation of the patient with an atrioventricular septal defect,1−5 such that in many instances this modality has been the sole imaging technique used to formulate a medical or surgical plan6. With the advent of Doppler echocardiography additional hemodynamic information is now available, which enables the cardiologist and surgeon to evaluate the site and magnitude of associated atrioventricular valvar regurgitation, and obtain an accurate measurement of pulmonary arterial pressure along with an assessment of outflow tract obstruction, if present.
Doppler derived systolic pressure gradients have become widely applied as noninvasively obtained estimates of the severity of aortic valvar stenosis. There is little correlation, however, between the Doppler derived peak instantaneous gradient and the peak-to-peak gradient obtained at catheterisation, the latter being the most applied variable to determine severity in children. The purpose of this study was to validate a mathematical model based on data from catheterisation which estimates the peak-to-peak gradient from variables which can be obtained by noninvasive means (Doppler derived mean gradient and pulse pressure), according to the formula: peak-to-peak systolic gradient=6.02+1.49*(mean gradient)−0.44*(pulse pressure). Simultaneous cardiac catheterization and Doppler studies were performed on 10 patients with congenital aortic valvar stenosis. Correlations between the gradients measured at catheter measured, and those derived by Doppler, were performed using linear regression analysis. The mean gradients correlated well (y=0.67 × + 11.11, r=0.87, SEE=6 mm Hg, p=0.001). The gradients predicted by the formula also correlated well with the peak-to-peak gradients measured at catheter (y=0.66 × + 14.44, r=0.84, SEE=9 mm Hg, p=O.002). The data support the application of the model, allowing noninvasive prediction of the peak-to-peak gradient across the aortic valvar stenosis.
To assess the clinical impact of percutaneously implanted balloon expandable endovascular stents on patient management, we reviewed 20 children aged 14 days to 4.8 years (median 1.3 years) with residual vascular obstructions in the immediate postoperative period. Patients included 11 with pulmonary arterial stenosis, five with stenosis of venous pathways after a modified Fontan procedure or bidirectional cavopulmonary anastomosis, and four with a restrictive modified Blalock-Taussig shunt. Placement of the stent was optimal in 18 of 20 patients (24 of 26 Palmaz or Palmaz-Schatz implants). In two patients, rupture of the balloon resulted in malposition of the stent. Reoperation was avoided and symptomatic improvement was noted in 11 of 20 patients (55%), while procedural complications occurred in eight patients. Seven of 10 patients presenting with a low cardiac output syndrome died despite relief of the obstructions. This early experience supports consideration of the application of these devices in the management of significant vascular obstructive lesions in the immediate postoperative period, thus avoiding early reoperation in this profoundly compromised population.
There is a clear and present danger for the patient considered for Fontan's operation in the presence of excessive ventricular hypertrophy and/or mass. Such ventricular hypertrophy and/or mass probably reflects singly, or in combination, banding of the pulmonary trunk and subaortic stenosis, as well as other factors. While one can construct a number of therapeutic algorithms for patients seen at various ages with a restrictive ventricular septal defect and subaortic stenosis in the setting of double inlet left ventricle and discordant ventriculoarterial connections, the optimal management should be to minimize the potential risks for development of subaortic stenosis and excessive ventricular hypertrophy and/or mass.
For the visualization of the coronary arterial patterns in complete transposition (concordant atrioventricular and discordant ventriculoarterial connections), balloon occlusion aortography has been performed in right and left anterior oblique or in frontal and lateral views. These views, however, are often unsatisfactory because of super-imposition of the aortic sinuses. The so-called “laid-back” view is a newly developed angiographic projection in which the aortic sinuses and the intervening commissures are imaged as if they were seen from below and the front. We performed such laid-back aortography in 36 consecutive patients with an echocardiographic diagnosis of complete transposition. In 23 of 36 patients, we obtained additional aortograms in right and left anterior oblique views. For the evaluation of the diagnostic value of the laid-back view, as compared with that of the combined right and left anterior oblique views, both sets of aortograms were reviewed independently by the five reviewers who were asked to make a diagnosis and to specify their levels of confidence (“definite,” “probable,” and “possible”) for their diagnosis. Error rates were significantly lower for the laid-back aortograms than for the combined right and left aortograms (4 and 11%, respectively). An increased level of confidence was significantly associated with the laid-back aortograms. We illustrate the anatomy of various coronary arterial patterns seen by laid-back aortography.
Bilateral arterial ducts represent an uncommon form of pulmonary or systemic blood supply associated with complex congenital cardiac disease. We sought to determine the role of high-resolution cross-sectional echocardiography and color flow Doppler ultrasonography in assessing this condition, as well as to describe the management and outcome in a group of patients. A retrospective review was conducted of 11 newborns identified over a 16-year period as having bilateral arterial ducts. Pulmonary atresia associated with non-confluent pulmonary arteries was the dominant lesion, with the heterotaxy syndrome also frequently being recognized. Echocardiography best identified the source of blood supply to either the pulmonary or systemic circulations, allowing differentiation from collateral vessels. Stenosis of the right or left pulmonary artery at the initial site of ductal insertion needs careful evaluation on follow-up. Management of patients with this condition remains a challenge, as indicated by the poor outcomes observed in our series.
A left superior caval vein frequently occurs in the malformed, as well as in the structurally normal, heart. Its physiological impact varies, and is determined by its connections and whether there are associated cardiac lesions. In this review, we describe 3 patients with such a vein, 1 without other lesions and 2 with the anomalous venous channel as a component of a complex cardiac malformation. In all cases, transcatheter techniques were used to treat the physiological dysfunction caused by the presence of the vein. The connections of the vein, and the complexity of the associated cardiac lesions, determine the options for treatment. We define the role of surgical as opposed to transcatheter intervention, and discuss strategies for transcatheter treatment.