Interruption of the aortic arch and malformative cardiac lesions requiring repair under extracorporeal circulation. Apropos of 3 cases

Interruption of the aortic arch is practically always associated with intracardiac malformations of variable complexity, at the least, a ventricular septal defect. Surgery is usually performed in two stages: aortic repair and pulmonary artery banding after intravenous prostaglandin administration. The second stage comprises debanding and repair of the intracardiac lesions under cardiopulmonary bypass. However, in some cases, interruption of the aortic arch is associated with intracardiac lesions which necessitate correction under cardiopulmonary bypass from the onset, this was the situation in two of the three cases described by the authors: aorto-pulmonary window, a lesion which can only be corrected under circulatory arrest and deep hypothermia. One of these two children, operated in the neonatal period, did not survive: the other, operated at 6 weeks, had an excellent result. In the third case, the association of tricuspid atresia and a restrictive ventricular septal defect necessitated enlargement of the septal defect and therefore, open heart surgery under circulatory arrest; the results were favourable.     

Enlarging angioplasty of the aortic arch with an pulmonary artery autograft patch. Interest in a single-stage repair of the syndrome of coarctation and interruption of the aortic arch]

Single stage repair of syndromes of coarctation and interruption of the aortic arch is a routine procedure in many surgical centres with good immediate results. The classical technique of aortic repair is based on the principles of Crafoord's extended resection anastomosis. Recoarctation is not an unusual long-term complication. A technique of enlarging angioplasty of the aorta using a patch of pulmonary artery has been developed and used in 22 neonates with obstruction of the aortic arch associated with ventricular septal defect with an average age and body weight of 15 days and 2.9 Kg respectively. The ventricular septal defect was closed surgically during the same procedure. Total circulatory arrest was not used in these children and all had aortic repairs with selective cerebral perfusion with moderate hypothermia (28-30 degrees C). This technique was used without any procedure-related early morbidity. No early or late deaths were observed in this series. Two patients were reoperated during the first year after the initial procedure: one for residual ventricular septal defect and the other for supraventricular pulmonary stenosis. Two patients, one of whom was reoperated, developed supraventricular pulmonary stenosis with a gradient of over 60 mmHg. These stenoses were observed in the first cases operated and were essentially due to the technique of pulmonary artery reconstruction. Over a median follow-up period of 10 months, no recoarctations were observed: the Doppler ultrasound study showed an isolated mean systolic gradient of 6 +/- 12 mmHg. The authors conclude that angioplasty of the aortic arch with an enlarging patch of pulmonary artery autograft during single stage surgery of syndromes of coarctation and interruption of the aortic arch provides a harmonious and durable repair of the aortic arch.     

Cardiac malformations in the velocardiofacial syndrome

A similar pattern of congenital anomalies found in 27 children, 9 male and 18 female, aged 2 weeks to 17 years, allows the conclusion that they represent a newly recognized malformation syndrome. All patients had velopharyngeal insufficiency, a submucous or overt cleft of the secondary palate and learning disabilities. A similar facies, characterized by a long vertical face, a large fleshy nose with broad nasal bridge, flattened malar region, narrow palpebral fissures and deep overbite with retruded mandible, was usually present. Other noncardiac anomalies also occurred frequently. Twenty-three (85 percent) had congenital cardiovascular malformations whose occurrence as single or combined lesions far exceeded the expected incidence in children with congenital heart disease. A ventricular septal defect was present in 15, tetralogy of Fallot in 5. Prolapse of the right aortic cusp was present in two patients with a ventricular septal defect, and aortic valve disease with insufficiency in one patient with tetralogy. The aortic arch was right-sided in 12 patients, in conjunction with a ventricular septal defect in 5, in association with tetralogy of Fallot in 4 and in the absence of associated cardiac disease in 3. The right aortic arch descended on the right in 10 patients; in 2 with a ventricular septal defect it crossed retroesophageally to descend on the left, and in one of these it presented as the “third aortic arch syndrome.” Aberrant origin of the left subclavian artery was present in five patients with a right aortic arch. A patent ductus arteriosus had caused congestive heart failure in infancy in one patient with a small ventricular septal defect; three others with a ventricular defect had additional clinically minor anomalies. Two children were brother and sister. Their mother and the mothers of two other children showed evidence of the same syndrome, suggesting the possibility of autosomal dominant or X chromosome-linked dominant transmission.     

One-stage repair of cardiac and arch anomalies without circulatory arrest

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1-43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.     

Intrapulmonary channel for one-stage correction of aortic arch obstruction

There are several methods of surgical repair of aortic coarctation or interruption; the optimal technique is still controversial. The purpose of this study was to assess a new surgical method: intrapulmonary channel for one-stage repair of aortic coarctation or interruption associated with intracardiac anomalies. Between 1993 and 1995, 4 patients with aortic coarctation or interruption and intracardiac anomalies received one-stage surgical correction. Their ages ranged from 5 to 26 years (mean, 16 years). The aortic arch lesions were preductal coarctation in 2, and type B interruption in 2. Coexisting anomalies consisted of patent ductus arteriosus in 4, ventricular septal defect in 3, and aortopulmonary window in 1. An intrapulmonary channel was constructed in all patients, and co-existing anomalies were corrected simultaneously. There was no hospital death or late mortality. A cerebral complication occurred in one patient because of air embolism. Mean follow-up was 9.5 years (range, 8.5-11.5 years). There was no evidence of recoarctation or late aneurysm formation. For selected patients with aortic coarctation or interruption and intracardiac anomalies, an intrapulmonary channel might be an option for one-stage correction.     

主动脉弓中断伴主肺动脉窗外科治疗——附5例临床分析

目的:总结5例主动脉弓中断(IAA)合并主肺动脉窗(APW)外科一期矫治术临床经验。方法:1997年12月至2009年12月共收治5例患儿,男性4例,女性1例。年龄1.7个月～13岁(中位年龄1.6岁),体质量4.7～26 kg。IAA A型3例,B型1例,C型1例;APW I型2例,Ⅲ型3例。合并心内畸形包括右肺动脉起源升主动脉、主动脉瓣下狭窄、二尖瓣关闭不全及房间隔缺损等。3例经未闭动脉导管置入灌注管,2例经升主动脉和股动脉插管建立体外循环。手术首先深低温下半身停循环,心包补片加宽或直接端端吻合重建主动脉弓;然后高位阻断升主动脉,心脏灌停后修补APW及其他心内畸形。结果:术后围术期死亡1例,其余4例顺利恢复出院,1例曾因肺部感染气管切开。平均ICU监护时间13.8 d,平均住院时间54.0 d。随访4个月～68个月(平均22个月),4例畸形均恢复正常,无1例主动脉吻合口狭窄,1例存在重度肺动脉高压,余3例肺动脉压力降至正常或接近正常。结论:IAA合并APW罕见,早期易产生肺动脉高压,尽早确诊一期矫治,18个月内手术,成功率高,远期预后良好。     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

Aortic coarctation in the first 3 months of life. Surgical results]

Between January 1973 and September 1989, 51 patients younger than 3 months with coarctation of the aorta underwent surgery. All of them had atrio-ventricular and ventriculo-arterial concordance with well developed ventricular cavities. Thirty-four were male and 17 female. Thirty five had associated anomalies and catheterism was done in 36 before surgical correction. The surgical procedures we used were 19 subclavian plasty (Waldhausen), 13 end-to-end anastomosis, 13 Alvarez technique and three goterex parch. Twelve died (23.5%), three during surgery and the others in a period of 3 to 20 days after surgery. Eight were younger than 17 days, seven had aortic arch hypoplasia associated and six had ventricular septal defect (five with pulmonary hypertension). Other ten developed recoarctation (gradient greater than 20 mmHg) between 10 days and 8 months after first intervention (media = 3 months). Five had previously end-to-end correction (41.6%), two angioplasty with parch (66%), two Alvarez (20%) and one Waldhausen (7%). The correction of the recoarctation required surgery in 4 patients (three with angioplasty with parch and one with end to end correction), and the other six underwent angioplasty with catheter-balloon. None of the 15 patients without previous catheterism died, and neither did those who underwent surgery during the last 4 years. The associated anomalies required a second time surgery. We conclude that morbimortality is related to the aortic arch hypoplasia, pulmonary hypertension and surgery during the first 2 weeks. We recommend surgery without previous catheterism. The recoarctation is more frequent in patients with end to end correction, without an increase of the mortality.     

Aortic coarctation in childhood. Surgical experience in 120 cases]

112 cases of coarctation of the aorta and 8 cases of tubular hypoplasia of the aortic isthmus operated upon in the Children's Hospital "La Paz" from Madrid, are reviewed. All children were under 7 years of age. 64.2% of the cases of aortic coarctation were in the first year of life, 47.3% of them had associated lesions, being the most frequently present persistent ductus arteriosus and ventricular septal defect. Hospital mortality was 14.2%, what is considered as very acceptable. All the children operated upon for the correction of tubular hypoplasia of the aortic isthmus were in the first year of age. 75% of them had associated ductus arteriosus and ventricular septal defect, being hospital mortality of 62.5%. Most frequent postoperative complications and cause of death were due to broncopulmonary disorders secondary to the existence of a previous pulmonary hypertension.     

Congenital heart defects and chromosomal anomalies including 22q11 microdeletion (CATCH 22).

OBJECTIVE: To analyze the frequency of chromosomal anomalies or 22q11 microdeletion in patients with congenital heart defects and other congenital anomalies; to describe the clinical phenotype of children with the 22q11 microdeletion and with chromosomal anomalies; to evaluate patients' clinical evolution; and to provide genetic counseling for families. METHODS: The study included 46 patients with congenital heart defects and other anomalies and patients with a phenotype consistent with 22q11 microdeletion observed between 1999 and 2001. Confirmation of the heart defect was accomplished through echocardiography, magnetic resonance angiography or cardiac catheterization. Karyotyping with high resolution banding and detection of 22q11 microdeletion with FISH techniques were performed. We excluded patients with trisomy 21, 13 and 18, 45,X and deletion of 7q11.23. Patients with 22q11 microdeletion underwent immunology studies and evaluation of parathyroid function. Clinical evolution was evaluated. Chromosome and FISH studies were performed on parents of affected children (25 couples). RESULTS: Forty-six children were included, of whom twelve (26.1%) had chromosomal anomalies (group A), fourteen (30.4%) had 22q11 microdeletion (group B) and the remaining twenty (43.5%) had normal karyotype and negative FISH studies (group C). In group A septal heart defects predominated. This group had significant morbidity, with surgical correction in three patients, early development of pulmonary hypertension, failure to thrive and serious neurological problems. Two patients died. In group B conotruncal heart defects (7/14) and ventricular septal defects (5/14, two associated with cervical aortic arch) predominated. The most significant morbidity was related to cardiac pathology, with surgical correction in seven cases (50%). Immune function defects and parathyroid function problems were mild, requiring no therapeutic measures. One patient died. CONCLUSION: In the presence of heart defects associated with other congenital anomalies, karyotyping is mandatory and if clinical features are compatible, 22q11 microdeletion should be specifically sought with FISH techniques. Detection of chromosomal anomalies has a significant impact on prognosis and follow-up of patients, as well as on genetic counseling of families.     

Interruption of the aortic arch in adults.

The interruption of the aortic arch is a rare cardiac congenital disease; such patients may occasionally survive to adulthood without surgery. The associated intracardiac malformations may modify survival. Our aim is to report three young adults (18-19 years of age) with interruption of the aortic arch. Two had type C, and the other had type B with subpulmonic ventricular septal defect and pulmonary valve insufficiency. We review 106 cases collected from the medical literature into 3 groups: 1) the whole group of patients; 2) patients with isolated interruption of the aortic arch, and 3) patients with interruption of the aortic arch associated with ventricular septal defect. In the whole group we found 18 cases of interruption of the aortic arch type A, and 25 cases of interruption of the aortic arch type B; 37 cases of isolated interruption of the aortic arch and 43 cases associated with ventricular septal defect. Fifty percent of the patients died before 15 days of life (0.042 years). According to the cumulative frequency graphic, only 5% of the patients survived beyond the age of 5 years. We found no information to relate patient's survival rate and anatomic type of the interruption of the aortic arch. From adolescence, the survival of the patients with interruption of the aortic arch associated to septal ventricular defect was 7%, and a 14% survival was found in patients with isolated interruption of the aortic arch. No statistical difference was found between the means of the ages of these two groups (P > 0.25).     

主动脉弓阻塞合并心脏畸形18例的一期矫治

目的总结一期矫治主动脉弓阻塞合并心脏畸形的诊治经验。方法2005年1月至2006年6月一期矫治18例主动脉弓阻塞合并心脏畸形。其中主动脉弓中断(IAA)6例,主动脉缩窄(COA)12例。手术均为正中开胸低温体外循环下一期畸形矫治术。结果手术采用降主动脉与主动脉弓端端吻合11例,端侧吻合6例,Gore-Tex片加宽弓成形1例。围术期死亡1例,总死亡率5.56%。喉返神经损伤2例,术后左主支气管受压1例。随访1～11个月,1例上下肢压差25mmHg,6例压差小于20mmHg,7例端侧吻合无压差。存活者无症状,生长发育良好。结论正中开胸一期矫治COA及IAA合并心脏畸形可获得满意的近期疗效。手术成功的关键是充分游离降主动脉,尽可能行端侧吻合术。     

Aortopulmonary window: clinical assessment and surgical results

INTRODUCTION AND OBJECTIVES: Aortopulmonary septal defect is an uncommon congenital cardiac anomaly. To date, approximately 300 cases have been reported. We present our experience, emphasizing the importance of early correction to avoid irreversible pulmonary hypertension. PATIENTS AND METHOD: Between 1979 and 2000, seven patients underwent surgical repair of this heart defect in our hospital. Two had type I (proximal), 4 had type II (distal) and 1 had type III (complete). Complex associated cardiac anomalies were present in 4 cases: type A interruption of the aortic arch in 2 cases, hypoplastic aortic arch in 1 and transposition of great arteries with ventricular septal defect in 1.Four cases (57%) were diagnosed by echocardiography. In all patients diagnoses were confirmed by cardiac catheterization.Patient records were reviewed retrospectively, with special attention to clinical, echocardiographic and hemodynamic data as well as surgical characteristics. RESULTS: No intraoperative deaths occurred. The patient with associated transposition of great arteries died 22 days after surgery as a result of severe pulmonary hypertension. The remaining patients are asymptomatic without treatment after a mean follow-up period of 69 months. CONCLUSIONS: Even though aortopulmonary septal defect is a rare anomaly, it should be considered whenever the course of complex congenital heart disease includes early cardiac failure and pulmonary hypertension. Repair before 6 months will prevent irreversible damage of pulmonary vessels.     

Determinants of survival following repair of interrupted aortic arch in infancy.

Between January 1971 and March 1987, surgery was performed in 26 infants with interrupted aortic arch. At operation the 14 boys and 12 girls weighted between 1.71 and 4.23 kg (mean +/- SD = 3.1 +/- 0.63 kg) and ranged in age from 2 to 90 days (13 +/- 18 days). The interruption was distal to the left subclavian artery in 4 (15%), between the left carotid and subclavian arteries in 20 (77%) and between the brachiocephalic (innominate) and left carotid arteries in 2 (8%). Associated complex cardiac lesions in 8 patients included complete transposition (2), common arterial trunk (2), aortopulmonary window (2), double inlet left ventricle (1) and tricuspid atresia (1). The remaining patients had an isolated ventricular septal defect. The arch was reconstructed with a prosthetic conduit in 14 patients; by a direct anastomosis in 6; using the subclavian artery in 3; and with the pulmonary trunk and the arterial duct in 2. Twenty patients (77%) underwent palliative surgery as the first stage of management, and banding of the pulmonary trunk was also performed in 16 of these. Five patients (19%) underwent primary complete repair of the interruption and intracardiac anomalies. One patient (4%) died soon after thoracotomy for palliative surgery. Of the 15 (57%, 70% confidence limits CL = 46-69%) early deaths, 7 occurred in patients with complex associated defects and 4 occurred when single stage repair was attempted. Survival following first-stage palliative surgery for arch interruption with isolated ventricular septal defect was 64% (9/14) [70% CL = 47-79%]. All of these patients subsequently underwent complete repair. Chi-squared and t-tests showed the year of operation and the type of operation (two-stage repair) to be associated with improved survival. It is concluded that a two-stage repair of interrupted aortic arch offers a reasonable alternative to primary complete correction and will lead to satisfactory subsequent repair in most cases.     

Surgical management of severe aortic outflow obstruction in lesions other than the hypoplastic left heart syndrome: use of a pulmonary artery to aorta anastomosis.

Between December 1985 and April 1990, 50 infants with a variety of congenital cardiac lesions other than the hypoplastic left heart syndrome underwent surgical relief of aortic outflow obstruction by creation of a pulmonary artery to aorta anastomosis. The patients were grouped anatomically by ventriculoarterial alignment. Nineteen had normally aligned great arteries (group I); 25 had transposition of the great arteries, all with a univentricular heart of left ventricular morphology (group II); and 6 had a double-outlet right ventricle (group III). All patients had either aortic stenosis with atresia, subaortic stenosis or a restrictive ventricular septal defect. Sixteen had normal arch anatomy; 34 had arch anomalies consisting of arch hypoplasia (n = 17), coarctation (n = 11), interruption of the arch (n = 4) and complex arch anomalies (n = 2). Surgery was performed at a median age of 10 days (range 2 to 184). Of the 50 infants, 33 survived. No significant difference in early survival (30 days) was noted among the groups of varying ventriculoarterial alignment (68% group I, 72% group II, 83% group III) (p greater than 0.05). Overall actuarial survival was 63% at 18 months. Analysis of actuarial survival by arch anatomy, although not statistically significant, revealed a trend toward better survival at 18 months postoperatively in infants with normal arch anatomy (81%) than in infants with arch anomalies (54%). Of the 33 survivors, 26 have proceeded to the next surgical stage, including the Fontan procedure in 8, superior cavopulmonary anastomosis in 13 and biventricular repair in 5.(ABSTRACT TRUNCATED AT 250 WORDS)     

Echocardiographic assessment of interrupted aortic arch

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.     

Diagnostic Role of Magnetic Resonance Imaging in Identifying Aortic Arch Anomalies

Purpose. The aim of this article was to assess the role of MRI in the diagnosis and management of a variety of complex aortic arch anomalies. Materials and Methods. Imaging was performed on a 1.5T Philips Gyroscan Intera. We retrospectively reviewed all cardiac MR scans performed from November 2003 to February 2007 at our institute to identify aortic arch anomalies. Magnetic resonance imaging included three‐dimensional (3D) sagittal volume images, cine images, short axis ventricular volumes, phase contrast flow of great vessels, and 3D gadolinium magnetic resonance angiogram. Results. Three major categories of anomalies were identified in 47 patients: double aortic arch (n = 9), right‐sided aortic arch with mirror image branching (n = 28), and aberrant right subclavian artery (n = 10). In the double aortic arch group, six patients were symptomatic, and four of them underwent a surgical division. Magnetic resonance imaging was used to plan the surgical management. In the other two groups, no patient presented with symptoms of airway or esophageal compression, and the arch abnormalities were noticed during investigation for other associated cardiac disease. However, we noticed a strong correlation with well‐defined subgroups of congenital heart disease. Right‐sided aortic arch was seen in patients with pulmonary atresia with ventricular septal defect (46.4%), tetralogy of Fallot (32.1%), and double outlet right ventricle with right atrial isomerism (14.2%). Seventy percent of the patients with aberrant right subclavian artery had aortic coarctation, and another case presented a complete aortic arch interruption. Conclusion. Magnetic resonance imaging is an important diagnostic tool in identifying anomalies of the aortic arch and its branches and can be considered the imaging technique of choice when planning surgical management, especially when there are associated cardiac anomalies.     

Cardiovascular anomalies in DiGeorge syndrome and importance of neural crest as a possible pathogenetic factor

One hundred sixty-one cases of DiGeorge syndrome (111 previously reported in which details concerning individual patients were given and 50 observed) were analyzed for occurrence and type of cardiovascular anomalies. Only 5 patients had a normal heart. Interrupted aortic arch type B was the major anomaly in 48 patients and persistent truncus arteriosus in 37. Therefore, in about half of the patients with DiGeorge syndrome the major anomaly was one that is rare. Conversely, of those patients with interrupted aortic arch, 68% had DiGeorge syndrome, as did 33% of all patients with truncus arteriosus. Although tetralogy of Fallot was also seen often in DiGeorge syndrome (10 patients), these cases represented less than 2% of the total number of cases of tetralogy of Fallot. Similarly, less than 1% of children with isolated ventricular septal defect or transposition of the great arteries had DiGeorge syndrome. The primary cardiovascular anomaly always involved the aortic arch system or the arterial pole of the heart. Recent studies show that neural crest cells play a crucial role in development of pharyngeal (bronchial) pouch derivatives, e.g., thymus and parathyroid glands, as well as the aortic arches and the truncoconal part of the heart. These studies and present observations support the view that DiGeorge syndrome and the associated cardiovascular anomalies are due to an abnormal developmental process involving the neural crest. Curiously, no instances of aortopulmonary septal defect or anomalous origin of a pulmonary artery from the ascending aorta (hemitruncus) have been associated with DiGeorge syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)     

Heart malformations in trisomy 13 and trisomy 18]

Congenital heart diseases were studied in children diagnosed of trisomy 13 and trisomy 18 in our hospital between January 1973 and July 1990. Twenty patients with trisomy 18 were diagnosed (18 females and two males). All had cardiac malformations. The findings were: ventricular septal defect in 16 cases (80%), valvular anomalies in 12 (63%), patent ductus arteriosus in nine (47%) and atrial septal defect or patent foramen ovale in 7 cases (36%). We found some complex congenital cardiac diseases: one atrioventricular canal, one tetralogy of Fallot, one hypoplastic left ventricle with mitral atresia and double outlet right ventricle, one case of univentricular heart with aortic outlet from a rudimentary cavity, a right ventricular atresia with pulmonary and tricuspid valves atresia. Nine cases of trisomy 13 were diagnosed (seven females and two males). We found: ventricular septal defect in 7 cases (77%), valvular disease in five (100% of the necropsy studies), secundum atrial septal defect in 4 patients (80%) and patent ductus arteriosus in two. Two cases presented hypoplastic left ventricle with aortic arch hypoplasia, one of them had subaortic stenosis and left superior vena cava being connected to the right atrium via coronary sinus; one case showed fibroelastosis. Our results have been similar to the previously reported and confirm the invariably presence of cardiac malformations in these syndromes. These malformations are an important sign of suspicion in fetal ultrasonography.     

Reabsorbable banding. Our initial experience]

Since May 1989 we have been using a reabsorbable 3 mm, polidioxanona band for pulmonary banding instead of the dacron one. This procedure was performed in 5 patients with ages between 33 to 230 days. The diagnoses were atrioventricular canal in one case and ventricular septal defect in the other 4 cases. One of them also had vascular ring with right aortic arch, left patent ductus arterious and aberrant subclavian artery. All the patients were in a poor condition. Subsequent reoperations performed in short, medium, and long term were uncomplicated, and the technique had the added benefit of avoiding the need for pulmonary reconstruction. A close ECO Doppler follow-up is necessary to determine the best moment for definitive correction. We think that this new technique makes the banding a reliable and complications free procedure and its place should be reevaluated in the light of the current tendency for early complete correction.