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PubMed1992

Truncus arteriosus communis associated with interrupted aortic arch: a report on two uncommon cases.

The paper presents two infants with the A-4 type of truncus arteriosus communis (according to Van Praagh's classification). One patient who survived a surgical procedure demonstrated a rare variant of aortic arch interruption to the left off the left subclavian artery (type A according to Celoria and Patton), whereas the second presented an uncommon anomaly in which the right subclavian artery originated from the descending aorta with associated severe truncal valve incompetency. The authors describe the clinical picture along with the surgical treatment of the first infant who being six days old was subjected to a correction employing the wide patent ductus arteriosus to reconstruct the aortic arch, following the method described by Gomes and McGoon. Subsequently an aortic homograft was implanted in order to connect the right ventricle and the pulmonary artery.

Aortic Arch Syndromes↗
PubMed1983

Interrupted aortic arch--anatomical features of surgical significance.

Analysis of 26 autopsy specimens with interruption of the aortic arch has led to a clearer understanding of the vital importance of associated cardiovascular abnormalities. In all cases there was an associated patent ductus arteriosus "supporting" the distal systemic circulation. "Proximal" septal defects, 22 ventricular septal defects and 3 aorto-pulmonary windows, were found in all but one specimen. The infundibular component of the ventricular septum was displaced posteriorly and leftward in 9 hearts resulting in significant left ventricular outflow obstruction in 8. In one specimen the infundibular septum was displaced anteriorly giving the effect of a "Fallot type" obstruction to the right ventricular outflow tract. Abnormal ventriculo-arterial connections were found in 7 hearts; 2 with discordant connection, 2 with double outlet, and 3 with single outlet of the heart. In 6 specimens there was an aberrant origin of the right subclavian from the distal aorta passing behind the esophagus to the right side. Various other arterial abnormalities were identified including a truncus arteriosus with separate but "transposed" origins of the pulmonary arteries. In short, in these hearts there were no examples of the rare situation where aortic arch interruption exists in isolation. This study therefore makes it very clear that a detailed understanding of associated anatomical abnormalities must accompany any attempt at surgical correction. Certainly in many of these cases management of the associated lesion will present more of a challenge than the difficult problem of dealing with the interrupted arch itself.

Abnormalities, Multiple↗
PubMed2002

Homograft conduit failure in infants is not due to somatic outgrowth.

OBJECTIVE: It has been assumed that the need for homograft replacement is due to somatic outgrowth, but this has not been adequately studied. Our objective was to identify reasons for homograft conduit failure. METHODS: The records and imaging studies of 40 patients undergoing homograft conduit replacement of the right ventricular outflow tract from 1996 to 2000 were retrospectively reviewed. RESULTS: The majority of patients had a diagnosis of tetralogy of Fallot (n = 20) and truncus arteriosus (n = 13). The median age at the initial operation was 8 months (0.25-108 months). The initial homograft sizes ranged from 9 to 22 mm, and 28 conduits were of pulmonary origin. When comparing size of the initial homograft with patients' expected pulmonary valve diameter (z = 0), oversizing was noted to be +3 (range, 0.83-5.4). Median interval to conduit failure was 5.3 years (0.83-11.3 years). At homograft replacement, only 12 patients had an existing conduit that was 1 SD below the homograft conduit size needed (z < or = -1). Most conduits had important regurgitation, but this was rarely a primary reason for reintervention (n = 1). Reoperation was usually required for stenosis, with a median gradient of 53 mm Hg (20-140 mm Hg). Stenosis was further categorized angiographically as follows: homograft valvular stenosis (shrinkage; 21/40 [53%]), distal anastomotic stenosis (4/40 [10%]), conduit kinking (3/40 [8%]), sternal compression (3/40 [8%]), posterior shelf impingement (2/40 [5%]), and somatic outgrowth (3/40 [8%]). Replacement in 2 patients was for proximal hood aneurysm. Several patients (7/40 [18%]) had stenosis at multiple levels. The average decrease in conduit diameter was 47% (28%-73%). CONCLUSIONS: Somatic outgrowth is seldom a primary reason for homograft conduit replacement of the right ventricular outflow tract. The most common cause for failure is conduit obstruction with thickening and shrinkage at the annular area. Conduit stenosis was responsible for failure in 53% of patients, technical issues were responsible for 30%, and only 8% failed as a result of somatic outgrowth. Placement of a smaller homograft (z = 0) at the initial operation may decrease the incidence of conduit kinking, sternal compression, and posterior shelf impingement.

Aortic Valve↗
PubMed1989

Anaesthetic management for caesarean section in a patient with uncorrected truncus arteriosus.

We describe the successful management of a 25-yr-old primigravida with uncorrected truncus arteriosus, requiring an urgent Caesarean section for delivery of a live infant and we discuss the rationale of using the chosen drug combination and the importance of adequate monitoring in selecting an anaesthetic technique based on the pathophysiology of the congenital cardiac lesion.

Adult↗
PubMed1984

Surgical treatment of truncus arteriosus in the first 6 months of life.

One hundred six infants were seen at the University of California Medical Center between 1974 and 1981 with the diagnosis of truncus arteriosus. One hundred of these underwent physiologic correction prior to 6 months of age. Six infants died prior to operation while undergoing intense medical therapy to improve their basic condition. There were 11 operative deaths with a mortality rate of 11%. Of the 86 long-term survivors, 55 have returned for conduit change because of either body growth or pseudointima proliferation of the conduit. There had been no mortalities at the time of conduit change, and 29 of these were repaired using a straight tube between the ventricle and pulmonary trunk, while 26 had valve conduits placed. Physiologic correction in the first 6 months of life has been accomplished with a low mortality rate and apparent good long-term results with none of the survivors having evidence of elevated pulmonary vascular resistance.

Blood Vessel Prosthesis↗
PubMed1989

Pulmonary artery banding revisited.

Pulmonary artery (PA) banding to reduce pulmonary blood flow was described by Muller and Dammann in 1952. This review describes the outcome of 170 children who had PA banding at the University of Virginia Medical Center between 1955 and 1988. One hundred and one of the patients were banded between 1958 and 1970; fewer bands were placed in later years because early total correction was feasible for certain conditions. When analyzed by preoperative diagnoses, the data reveal that children with a single ventricle undergoing banding had a significantly lower 30-day mortality rate of 12% compared to other preoperative diagnoses, including atrioventricular canal, truncus arteriosus, and ventricular septal defect (VSD) at 30% (p less than 0.05). The late overall mortality for all patients was approximately 10%, an attrition rate of 1% per year. PA banding still has a role in management of patients with congenital heart disease, particularly for infants with a single ventricle. Actuarial survival at 10 years for patients with this condition is 92%. Interestingly, this indication for pulmonary banding is the same one cited in the original report.

Aortic Coarctation↗
PubMed1985

Truncus arteriosus and facial dysmorphism.

Facial features of infants with truncus arteriosus were photographed and reviewed. Anomalies included hypertelorism, low set ears, micrognathia, down-slanting palpebral fissures, short philtrum and small mouths. Associated cardiovascular defects were interrupted aortic arch, double aortic arch, right aortic arch and aberrant brachiocephalic vessels. Potential teratogenic factors included maternal diabetes, syphilis, alcohol ingestion, carbimazole therapy and infant chromosomal anomalies. The facial features, together with the rare cardiac abnormalities of truncus arteriosus and aortic arch defects are similar to those described in Di George syndrome (defect of fourth branchial arch and derivatives of third and fourth pharyngeal pouches). However none of these patients had symptomatic hypocalcaemia or absence of the thymus. It is concluded that this association of truncus arteriosus, aortic arch abnormalities and facial anomalies involves first and fourth branchial arch maldevelopment, and indicates embryological insult between the fourth and seventh weeks of gestation.

Abnormalities, Multiple↗
PubMed1991

Transesophageal echocardiography in aortopulmonary communications.

The assessment of congenital aortopulmonary communications by transthoracic echocardiography may be suboptimal, particularly postoperatively, due to limited acoustic windows. We performed intraoperative transesophageal echocardiograms in six patients with eight proven systemic-pulmonary communications. Diagnosis included truncus arteriosus (1), aortopulmonary window (1), Waterston anastomosis (3), central Gore-Tex shunt (1), and modified left Blalock-Taussig shunt (2). All communications were accurately demonstrated by transesophageal echocardiography (TEE). The transesophageal technique also provided an assessment of the size of the aortopulmonary communications and the proximal pulmonary arteries. In addition, the gradient across some of the communications could be accurately estimated utilizing the high pulse repetition frequency Doppler. On the other hand, only two of the seven aortopulmonary communications were detected by transthoracic echocardiography. Postoperatively, transesophageal imaging demonstrated unobstructed conduit in five of five patients who underwent conduit repair, as well as intact closure of aortopulmonary communications and concomitant closure of ventricular septal defects.

Aortopulmonary Septal Defect↗
PubMed1987

Surgical repair of hemitruncus: principles and techniques.

Ten patients have presented at the World Children's Hospital of Melbourne with hemitruncus, a form of truncus arteriosus. Nine of these patients underwent successful one- or two-stage hemodynamic repair. Early repair is carried out to avoid pulmonary vascular disease of the lung directly supplied from the trunk and to recruit as much of the contralateral pulmonary artery vasculature bed as possible is a secondary goal. The repair should be contemplated within the first six months of life to prevent severe pulmonary vasculature obstructive disease. Anatomy that is repairable in one stage is when the left pulmonary artery originates from the trunk and the complete right pulmonary artery supplied by pulmonary collaterals from the arch to the descending aorta. Anatomy requiring two-stage repair occurs when the RPA rises from the trunk and the left PDA rises from the descending aorta or distal aortic notch and either the right or the left lung blood supply is from multiple origins and requires unifocalization. With these anatomical principles and the general meticulous perioperative and postoperative care of the infants, a high degree of success should be obtainable with repair of this hemitruncus.

Child, Preschool↗
PubMed1990

Repair of truncus arteriosus and interrupted aortic arch.

A total of seven patients with truncus arteriosus and interrupted aortic arch (IAA) comprises our surgical experience in this condition. All underwent primary complete repair via median sternotomy between June 1985 and December 1989. Median age at repair was 8 days and median weight, 3.2 kg. Anatomy of these seven patients was truncus arteriosus type "1 1/2" in five patients and type II in two patients, IAA type B in six patients and type A in one patient. Aortic arch was reconstructed by direct anastomosis of ascending aorta and descending aorta. Right ventricle to pulmonary artery continuity was established with a porcine valved conduit in four patients, aortic homograft in two, and aortic homograft monocusp patch in one. Three patients have required five reoperations (three in one patient). One reoperation was due to compression of the left main bronchus from the reconstructed aorta, one was due to obstruction of the aorta at the site of IAA repair, and one was due to compression of the left main bronchus, right pulmonary artery, and residual stenosis across the hypoplastic ascending aorta. There were no early or late deaths and all seven survivors are currently well with a mean follow-up of 29 months from initial repair.

Anastomosis, Surgical↗