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PubMed1979

Discontinuity between the heart and the pulmonary circulation. Its management with the use of a valved external conduit.

The Hancock conduit that contains a porcine xenograft valve has been used in part of the cardiac repair of 22 patients with complex congenital heart disease. Five patients had a severe form of tetralogy of Fallot; six had pulmonary atresia; five had transposition of the great vessels, ventricular septal defect (VSD), and pulmonic stenosis; five had truncus arteriosus; and one had "corrected" transposition, VSD, and pulmonic stenosis. The hospital mortality was 2/22. This conduit has proved a satisfactory method to establish right ventricular-pulmonary artery continuity.

Bioprosthesis↗
PubMed1996

Truncus arteriosus malformation: a developmental arrest at Carnegie stage 14.

The pathogenesis of truncus arteriosus malformation has been ascribed to deficiency of conotruncal ridges, failure to form a pulmonary conus, absence of the aortopulmonary septum, or hemodynamic factors. To re-examine this issue, we reviewed the morphology of 28 hearts with truncus arteriosus malformation and compared the findings to the sequences of cardiogenesis in 351 normal human embryos of the Carnegie Embryological Collection. All malformed hearts had an absent muscular outflow tract (conal) septum. The truncal valve had four commissures and/or raphes, a fused commissure, in seven cases, three in 20, and uncertain status in one. All but one heart had fibrous continuity between the anterior mitral leaflet and the truncal valve. In embryos, the outflow tract was circular and lined by a layer of cardiac jelly early in Carnegie stage 14 but acquired an elliptical configuration and four cushions, with fusion of the two larger, by stage 16. Semilunar valve leaflets form at the downstream end of the cushions. The more frequent occurrence of three rather than four commissures in the truncal valve associated with absence of a conal septum suggests that the embryonic outflow tract failed to acquire an elliptical shape and cushions that could fuse to subdivide the outflow tract and the semilunar valves anatomically. The presence of truncal valve leaflets shows that three or four cushions did form and the usual presence of mitral-truncal valve fibrous continuity supports otherwise normal outflow tract development. We conclude that a failure or delay of the circular outflow tract of early Carnegie stage 14 to acquire the elliptical configuration needed to induce formation of four cushions, two of which fuse to subdivide the outflow tract and semilunar valve primordia, is the cause of truncus arteriosus malformation.

Adolescent↗
PubMed2000

Left hemitruncus associated with tetralogy of fallot: a case report.

This report presents a case of a left hemitruncus and tetralogy of Fallot. The diagnosis was first made by means of echocardiography and confirmed with cardiac catheterization thereafter. To the authors' knowledge, this patient is the fourth-longest-term survivor so far reported in the medical literature. A significant improvement has been observed after surgical correction of the anomaly, and the patient was free of symptoms at the 6-mo follow-up examination.

Abnormalities, Multiple↗
PubMed1982

Brief clinical report: deletion of the long arm of chromosome 11, [del(11)(q23)].

We report sporadic occurrence of deletion of the long arm of chromosome 11 (q23 leads to qter) in a male newborn infant with intrauterine growth retardation, craniofacial, cardiac, and orthopedic abnormalities and neonatal death but without genital abnormalities. This deletion is seen predominantly in females; here we emphasize the importance of an XX sex chromosome constitution as a factor determining phenotypic expression of and survival in the del(11q) syndrome. We also provide a description of the cardiovascular system from postmortem examination. The cardiac findings are similar to those of two previously autopsied cases and will assist in early clinical diagnosis of the 11q-syndrome.

Abnormalities, Multiple↗
PubMed2005

Coronary artery embryogenesis in cardiac defects induced by retinoic acid in mice.

BACKGROUND: Although normal coronary artery embryogenesis is well described in the literature, little is known about the development of coronary vessels in abnormal hearts. METHODS: We used an animal model of retinoic acid (RA)-evoked outflow tract malformations (e.g., double outlet right ventricle [DORV], transposition of the great arteries [TGA], and common truncus arteriosus [CTA]) to study the embryogenesis of coronary arteries using endothelial cell markers (anti-PECAM-1 antibodies and Griffonia simplicifolia I (GSI) lectin). These markers were applied to serial sections of staged mouse hearts to demonstrate the location of coronary artery primordia. RESULTS: In malformations with a dextropositioned aorta, the shape of the peritruncal plexus, from which the coronary arteries develop, differed from that of control hearts. This difference in the shape of the early capillary plexus in the control and RA-treated hearts depends on the position of the aorta relative to the pulmonary trunk. In both normal and RA-treated hearts, there are several capillary penetrations to each aortic sinus facing the pulmonary trunk, but eventually only 1 coronary artery establishes patency with 1 aortic sinus. CONCLUSIONS: The abnormal location of the vessel primordia induces defective courses of coronary arteries; creates fistulas, a single coronary artery, and dilated vessel lumens; and leaves certain areas of the heart devoid of coronary artery branches. RA-evoked heart malformations may be a useful model for elucidating abnormal patterns of coronary artery development and may shed some light on the angiogenesis of coronary artery formation.

Animals↗
PubMed1980

Truncus arteriosus communis, an unusual anatomical variant of type I.

We present a case of type I truncus arteriosus communis characterized by the horizontal origin of the main pulmonary artery from the common trunk and by a winding course up to a total length of 11.5 cm to the point of bifurcation of the pulmonary artery. Other than cyanosis the patient had a normal clinical history, which was followed for 20 years. The length of the main pulmonary artery could be related to the lower site of the incomplete septation of the common trunk, and it seems that hemodynamic factors contributed to this unusual configuration.

Angiocardiography↗
PubMed1993

Contralateral femoral vein removal of a circumferentially ruptured balloon-catheter following pulmonary artery angioplasty.

While attempting to remove a circumferentially ruptured balloon following branch pulmonary artery angioplasty, the shaft of the catheter broke, leaving the distal portion of the balloon and catheter in the femoral vein but still on the exchange wire. We describe a method to retrieve the broken balloon catheter via the contralateral femoral vein.

Angioplasty, Balloon↗
PubMed2006

Prenatal diagnosis of an unexpected interstitial 22q11.2 deletion causing truncus arteriosus and thymic hypoplasia in a ring 22 chromosome derived from a maternally inherited paracentric inversion.

OBJECTIVE: To present the prenatal diagnosis of an interstitial 22q11.2 deletion involving a ring 22 chromosome associated with truncus arteriosus and a hypoplastic thymus. CASE: Following the sonographic diagnosis of a cystic hygroma at 12 weeks of gestation, chromosome analysis revealed a ring 22 chromosome. RESULTS: Ring chromosomes typically result in the deletion of genetic material from the distal long and short arms of the affected chromosome. The presence of an interstitial deletion in a ring chromosome is therefore unusual. FISH analysis revealed an unexpected deletion involving the TUPLE1 gene in the DiGeorge/Velocardiofacial syndrome region in 22q11.2. Maternal chromosome analysis revealed the cause of the apparent interstitial deletion, a paracentric inversion in the long arm of chromosome 22, resulting in the distal long arm of 22q being located adjacent to the centromere and the proximal end being located near the telomere. The fetus was subsequently diagnosed with truncus arteriosus and a hypoplastic thymus, consistent with DiGeorge syndrome. CONCLUSION: The ring chromosome 22 found in the fetus appears to have been derived from a rearrangement of the mother's inverted 22, resulting in ring formation and loss of the end of the distal long arm of the inverted 22, including the TUPLE1 locus, causing DiGeorge syndrome in the fetus. The apparent interstitial deletion was actually a terminal deletion in a maternally inherited rearranged chromosome 22.

Abnormalities, Multiple↗
PubMed2003

Prenatal diagnosis of mosaic ring chromosome 22 associated with cardiovascular abnormalities and intrauterine growth restriction.

OBJECTIVES: To present the prenatal diagnosis and perinatal findings of mosaic ring chromosome 22. CASE: Amniocentesis was performed at 18 gestational weeks because of an advanced maternal age. Cytogenetic analysis of the cultured amniotic fluid cells revealed mosaicism for ring chromosome 22, 45,XX,-22[6]/46,XX,r(22)(p13q13.31)[15]. Abnormal fetal sonographic findings included small for gestational age, a ventricular septal defect, and truncus arteriosus. The pregnancy was terminated. Additional phenotypic findings included hypertelorism, epicanthal folds, and abnormal ears. Cytogenetic analysis of the cord blood lymphocytes revealed a complex mosaic karyotype, 45,XX,-22[7]/46,XX,r(22)(p13q13.31)[82]/46,XX,idic r(22)(p13q13.31;p13q13.31)[11]. Cytogenetic analysis of the hepatocytes also revealed mosaic r(22) with mosaicism for idic r(22) and monosomy 22. The deletion of distal 22q and the duplication of 22q11.2 on idic r(22), and the distal 22q deletion on r(22) were demonstrated by fluorescent in situ hybridization (FISH) analysis using 22q terminal probes at 22q13 and a DiGeorge syndrome critical region probe at 22q11.2. The breakpoint on distal 22q13 and the extent of the duplication of 22q on idic r(22) was determined by examining polymorphic markers specific for chromosome 22 using quantitative fluorescent polymerase chain reaction assays. The chromosomal aberration was of maternal origin. CONCLUSION: Molecular and FISH studies allow a better delineation of some prenatally detected aneuploidy syndromes and help elucidate the genetic pathogenesis. Fetuses having mosaic r(22) with a low level mosaicism for r(22) duplication/deletion may present cardiovascular abnormalities and intrauterine growth restriction on prenatal ultrasound.

Abnormalities, Multiple↗
PubMed1980

Syndromes of cardiovascular malformations induced by copper citrate in hamsters.

Pregnant golden hamsters were injected intraperitoneally on the morning of the eighth day of gestation with a solution of copper citrate (2.7 mg/kg) or deionized water. Embryos were removed from the mothers by laparotomy on the 12th and 13th days of gestation, serially sectioned through the thorax, and examined for cardiac malformations. Twenty-one of the 37 edematous embryos from copper-treated mothers were affected with a total of 58 major cardiac anomalies. Sixteen of these embryos were affected with double outlet right ventricle in association with a membranous ventricular septal defect, two embryos contained hearts with overriding aortas, and three embryos were affected with isolated ventricular septal defects. Eleven of the 16 embryos with double outlet right ventricle were also affected with pulmonary trunk hypoplasia. The concept that these cardiac anomalies arise from a common pathogenetic basis is supported by the present model.

Animals↗