Ductus arteriosus dilatation by prostaglandin E1 in infants with pulmonary atresia

Infants with pulmonary atresia depend on patency of the ductus arteriosus for survival in the immediate postnatal period. Despite continuing hypoxemia after birth the ductus arteriosus usually constricts, thus reducing pulmonary blood flow. This often occurs while awaiting surgical palliation or correction, leading either to marked deterioration in the infant's condition, or death. In ten infants with pulmonary atresia, we infused prostaglandin E1 (PGE1) at a rate of 0.1 mug/kg/min in six and 0.05 mug/kg/min in four into the descending aorta at the orifice of the ductus arteriosus. The ductus arteriosus was effectively dilated; at the narrowest point the diameter, measured in eight infants, almost doubled. In all ten infants arterial blood PO2 increased, averaging 24.6 mm Hg before and 43.7 mm Hg after the infusion was started. Infusion of PGE1 directly into the aorta adjacent to the ductus arteriosus avoided the complications of pyrexia, muscular twitching, and excitability which may be related to the effects of prostaglandins on the central nervous system.     

Newborn with congenital arteriovenous fistulas of the abdominal wall complicated by a ductus arteriosus aneurysm

A case of congestive heart failure in a neonate resulting from a congenital abdominal arteriovenous fistula is described; an aneurysm of the ductus arteriosus was also found in the patient. Abdominal aortography was performed and demonstrated dilated feeding arteries, including the hypogastric arteries, which communicated with a dilated umbilical vein. Ligation of the feeding arteries and the umbilical vein led to resolution of the heart failure. Transient liver dysfunction occurred, however. Symptoms improved dramatically upon removal of the ligature from the umbilical vein. Although there is one previous report of an arteriovenous fistula involving the umbilical vein, we know of no prior report of a congenital arteriovenous fistula in association with a ductus arteriosus aneurysm.     

Debt and career choices of underrepresented minorities

We report a case of right aortic arch with mirror-image branching and coarctation of the aorta and left ductus arteriosus in a 6-year-old boy. He was referred to Chang Gung Children's Hospital with suspected left ventricular failure. Physical examination revealed hypertension and a pulse discrepancy between the upper and lower extremities. Echocardiography and magnetic resonance imaging revealed a right aortic arch, a long segmental aortic coarctation, and a patent ductus arteriosus. In addition, mirror-image branching of the brachiocephalic vessels was found. The coarctation was resected and the ductus arteriosus was ligated. The postoperative recovery was uneventful and the blood pressure returned to normal. He remained normotensive and had no symptoms of heart failure within the 2 years of follow-up.     

A quick mnemonic for predicting pressure sores in ED patients

We describe the first successful balloon angioplasty of a coarctation in a 460-g newborn infant with coarctation of the aorta and heart failure. A coronary angioplasty catheter was positioned across the coarctation via a transumbilical approach. The waist of the balloon disappeared on maximal inflation and there was an increase in blood pressure distal to the coarctation and the clinical status improved. A ductus arteriosus was ligated 4 days after angioplasty.     

Aneurysm of the nonpatent ductus arteriosus in the adult--a report of the case and review of the literature

Aneurysm of the ductus arterious in adult in rare. 28 cases have been previously reported in the Japanese literature. A new 73-year-old male case, preoperatively diagnosed and successfully repaired, are presented. The patient with hoarseness was admitted to our hospital. Contrast-enhanced CT scans and aortograms made a diagnosis for the aneurysm of the ductus arteriosus. Operation was done through left thoracotomy with the aid of partial cardiopulmonary bypass. The pulmonary arterial end of the ductus arteriosus was closed and a Ligamentum arteriosus was identified. A 3 by 2.7 cm saccular aneurysm was resected and segmental replacement of the aorta with woven dacron graft was performed. Because of it's critical location and the high incidence of complications, aneurysm of the ductus arteriosus in the adult should be surgically corrected when diagnosed.     

Microtubule-associated protein 1 light chain 3 is a fibronectin mRNA-binding protein linked to mRNA translation in lamb vascular smooth muscle cells

Intimal cushions form in the fetal ductus arteriosus by fibronectin-dependent smooth muscle cell migration which is associated with greater efficiency of fibronectin mRNA translation. We investigated whether the AU-rich element (ARE), UUAUUUAU, in the 3'-untranslated region (3'UTR) of fibronectin mRNA is involved in this mechanism by transfecting smooth muscle cells with plasmids containing the chloramphenicol acetyltransferase coding region with its 3'UTR replaced by fibronectin 3'UTR bearing intact or mutated ARE. More efficient translation of fusion mRNA with intact versus mutated ARE was observed. This effect was amplified in ductus (10.9-fold) compared with nonmigratory, lower fibronectin-producing aorta cells (6.5-fold). Ductus cells transfected with wild-type but not ARE-mutated plasmid reverted to the stellate phenotype of aorta cells associated with reduced fibronectin production. This suggested that plasmid ARE sequesters RNA-binding factors, thereby reducing endogenous fibronectin mRNA translation. We next purified a 15-kD fibronectin ARE-dependent RNA-binding protein and identified it as microtubule-associated protein 1 light chain 3 (LC3). LC3 is present in greater amounts in ductus compared with aorta cells, and overexpression of LC3 in aortic cells by transfection enhances fibronectin mRNA translation to levels observed in ductus cells.     

Echocardiographic aspects of the aortopulmonary window. Report of a case with angiocardiographic correlations

A case of complex congenital heart disease is presented with diagnosis of; ventricular septal defect, interrupt aortic arch, patent ductus arteriosus and aorticopulmonary window. The latter condition was studied by means of M mode and two-dimensional echocardiographic techniques. The heart was scanned perpendicular to its long axis at the origin of the great arteries. This scan revealed an image of two adjacent circular structures which has been previously described as suggestive of dTGA or Double-outlet right ventricle. The clinical picture discarded the possibility of dTGA, and the atrioventricular relationship established by the cross-sectional echocardiography invalidated the diagnosis of Double-outlet right ventricle. The cause of the echocardiographic morphologies observed are discussed.     

Lack of pharmacokinetic interaction between mexiletine and omeprazole

A neonate presented to our institution with the physical findings of coarctation of the aorta. After starting prostaglandin E1 the signs and symptoms resolved despite persistent closure of the ductus arteriosus. We present echocardiographic evidence to support the contention that a ductal tissue sling contributes to the formation of juxtaductal coarctation of the aorta.     

Association between parachute mitral valve and Down's syndrome. Report of a case

The trisomy 21 form of Down's syndrome is the most common human chromosomal aberration. Congenital heart disease is found in as many as 50 per cent of patients with this disorder. The two most common cardiac lesions in Down's syndrome are septal ventricular defect and endocardial cushion defect. Secundum atrial septal defect, tetralogy of Fallot and isolated patent ductus arteriosus are also observed in these Down's patients. Transposition of great arteries and coarctation of the aorta are rarely seen. Most patients having Down's syndrome with congenital heart disease have a single lesion. However, as many as 30 per cent may have multiple cardiac defects. Parachute mitral valve is a rare congenital mitral defect: a single papillary muscle in the left ventricle is the hallmark of this lesion. A parachute mitral valve is frequently associated with other left heart disorders such as supravalvular mitral ring, abnormal and stenosed mitral valve, subaortic stenosis and coarctation of the aorta, thus constituting either a complete form of Shone's complex (when all 4 components are present) or an incomplete form when there are fewer. The aim of the present report is to describe the connection between Down's syndrome and isolated, non-stenosed parachute mitral valve, which has never been reported before.     

Plug closure of patent ductus arteriosus by transfemoral catheter method. A comparative study with surgery and a new technical modification

Plug closure of patent ductus arteriosus without thoracotomy is reliable and can be a good alternative to surgical closure for selected patients. We report our experiences with plug closure in 87 consecutive patients (age range, 3 to 38 years). Closure was successful in 83 patients (95 percent). There has been no mortality, and the failure in four patients (5 percent) was mainly due to an oversized ductus. A major complication during the procedure was dislodgment of the plug into either the aorta or the pulmonary artery, which occurred in five patients (6 percent). The late results were quite satisfactory, with no recurrence of shunting or any other complications. Comparison of these results with those of 100 patients treated surgically showed that the new catheter method did not seem to carry a higher risk but had certain advantages over thoracotomy. A simpler and less time-consuming method using a single catheter has been devised and successfully used in the most recent nine patients without failure. Simplification of the technique has signficantly reduced the time of the procedure and the dose of radiation.     

Characteristics of a low affinity passive Ca2+ influx component in rat parotid gland basolateral plasma membrane vesicles

Prenatal detection of intrauterine closure of the ductus arteriosus unrelated to maternal administration of non-steroidal anti-inflammatory drugs or glucocorticoids made it possible to study the circulation in this condition in the human fetus and newborn by pre- and postnatal echocardiography and neonatal cardiac catheterization. At 38 weeks, the fetus presented intrauterine ductal closure associated with right ventricular dilatation and marked hypertrophy of the right ventricle and the interventricular septum, as well as severely diminished right ventricular fractional shortening and diminished pulmonary blood flow. Blood flow redistribution was characterized by reduced blood flow through the right heart and increased right-to-left shunting across the dilated foramen ovale. Pathological Doppler waveforms of the inferior vena cava and the ductus venosus were found, although the cardiotocogram was normal. Following unsuccessful induction of labour a Caesarean section was performed. Postnatal echocardiography confirmed the prenatal findings. Cardiac catheterization, performed because of persistent dependence on additional oxygen administration, revealed increased pulmonary vascular resistance, reduced pulmonary blood flow, and prolonged right-to-left shunt across the foramen ovale. Reduced peripheral pulmonary artery diameters were shown angiographically. Follow-up examinations revealed regression of right ventricular hypertrophy and recovery of right ventricular and pulmonary function. The findings confirm results from haemodynamic studies in animal experiments.     

Mechanisms of oxygen-induced contraction of ductus arteriosus isolated from the fetal rabbit

The present study was designed to investigate the effect of O2 on intracellular Ca concentration ([Ca]i) in the ductus arteriosus and the mechanisms for O2-induced ductal contraction. The force of isometric contraction of the ring of the ductus arteriosus isolated from fetal rabbits at 30 days of gestation (term, 31 days) was measured. The ductus arteriosus was loaded with fura 2, a calcium-sensitive dye, and [Ca]i was determined from the ratio of fluorescence intensity at 340 and 380 nm excitation wavelengths. The ductus arteriosus was initially superfused with hypoxic control solutions and contraction was induced by application of oxygenated solutions. The O2-induced contraction of the ductus arteriosus was associated with increases in [Ca]i and was eliminated in the absence of extracellular calcium. An increase in [K]o from 5 to 50 mM, which causes membrane depolarization, induced ductal contraction. The calcium channel blockers verapamil, diltiazem, and nickel caused a similar inhibition of O2-induced contraction as well as KCl-induced contraction. The role of intracellular calcium stores in O2-induced ductal contraction was examined using ryanodine, an inhibitor of calcium uptake and release from the sarcoplasmic reticulum. The inhibition of O2-induced contraction by ryanodine was minimal. Infusion of glibenclamide, an inhibitor for opening the ATP-sensitive potassium channel, caused contraction of the ductus arteriosus in the hypoxic solution. Cromakalim, an opener of ATP-sensitive potassium channels, completely relaxed the contraction induced by O2. These data suggest that O2 increases [Ca]i and causes contraction in the ductus arteriosus. Application of O2 may change from anaerobic to aerobic metabolism and depolarize membrane potential by closing the ATP-sensitive potassium channel, which in turn increases calcium influx via the voltage-dependent calcium channel. Mechanisms other than the ATP-sensitive potassium channel may also be involved in the O2-induced contraction and remain to be studied.     

Heterogeneity of clonal development in chronic myeloproliferative disorders

A 4-kg male child, born at 34 weeks to a gestational diabetic mother, had a large ductus arteriosus aneurysm causing phrenic and recurrent laryngeal nerve palsies and large airway compression. The right and left atrial appendages and distal descending aorta were cannulated, allowing left heart partial or complete cardiopulmonary bypass as necessary. On bypass the ductus was ligated, decompressed, and oversewn but not excised. Examination 1 month later suggested resolution of the recurrent laryngeal palsy and echocardiography showed regression of the aneurysm. Ductus ligation and decompression was an effective surgical treatment, which is less likely to cause complications than resection.     

A case report of one-stage repair for transposition of the great arteries with intact ventricular septum and coarctation of the aorta in neonate

We report a neonatal case of one-stage repair for transposition of the great arteries with intact ventricular septum (TGA with IVS) and coarctation of the aorta (CoA). The surgery was performed at 27 days of age when the patient, a male, weighed 3408 g. Preoperative cardiac catheterization and angiography confirmed the diagnosis of TGA with IVS, CoA, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava, left sided juxta-positioning of appendages and dextrocardia. The procedure was simultaneous subclavian flap aortoplasty through a left thoracotomy and an arterial switch operation through a median sternotomy. The patient's postoperative course was uneventful and he has grown well. Postoperative cardiac catheterization revealed mild pulmonary stenosis (pressure gradient of 19 mmHg between the right ventricle and the pulmonary artery) and mild aortic arch kinking from the arterial switch maneuver. One-stage repair, the combination of subclavian flap aortoplasty and arterial switch operation, provides a good clinical result in this complex malformation.     

Sex, birth order, and maternal age characteristics of infants with congenital heart defects

The records of the New England Regional Infant Cardiac Program, a service program covering all of New England, provide a useful source of information about the characteristics of children born with congenital heart defects. Data were analyzed on more than 2000 children born in New England who were diagnosed with a congenital heart defect before the first birthday. Children with arterio-venous fistula, aortic stenosis, transposition of the great arteries or hypoplastic left ventricle were predominantly male; children with persistent ductus arteriosus and endocardial cushion defect were predominantly female. Positive trends in risk with increasing birth order were present for pulmonic stenosis and transposition of the great arteries, and a negative trend was seen for persistent ductus arteriosus. What evidence there was for associations with maternal age was greatly reduced after controlling for confounding by birth order.     

Physiologic changes associated with ligation of the ductus arteriosus in preterm infants

Cardiorespiratory and transcutaneous oxygen monitors were used on 13 preterm neonates to examine physiologic changes during ductus arteriosus ligation. Transcutaneous oxygen decreased 30 seconds after left lung deflation; all infants required increases in inspired oxygen and ventilation to correct abnormal values after the left lung was compressed. Transcutaneous oxygen decreased 30 seconds after ductus arteriosus ligation (mean delta tcPO2 = -17 mm Hg +/- 11.4) but increased 150 seconds after left lung inflation (mean delta tcPO2 = 46.9 mm Hg +/- 28.8). Arterial blood pressure increased (mean delta systolic BP = 17.9 mm Hg +/- 9.5) and heart rate decreased 10 seconds after ductus arteriosus ligation. In five neonates, gradual closure of the ductus arteriosus over 40 to 75 seconds resulted in a more gradual increase in blood pressure. Intraventricular hemorrhage was confirmed in two patients after surgery. Ligation of the ductus arteriosus results in an abrupt increase in blood pressure, which may be related to the pathogenesis of intraventricular hemorrhage. We suggest that the ductus arteriosus be closed gradually to allow a more gradual increase in blood pressure.     

Controllable graded cerebral ischaemia in the gerbil: studies of cerebral blood flow and energy metabolism by hydrogen clearance and 31P NMR spectroscopy

Patent ductus arteriosus is an uncommon anomaly in adult patients. Surgical closure of patent ductus arteriosus in this age group presents difficult problems to the surgeon. We report our experience of 21 adult patients (19-62 years of age, mean 40 years) who underwent closure of the ductus by transfemoral implantation of a Rashkind double umbrella device. The patients came to light because of atrial fibrillation, congestive heart failure, residual flow after surgical ligation of the duct or because of incidental diagnosis made during physical examination or chest X-ray. In ten patients the pulmonary arterial pressure was normal (systolic pressure < 30 mmHg), in eleven it was elevated (systolic pressure from 30 to 100 mmHg, mean 50 mmHg). In seven patients the duct was clearly calcified and the size of the duct varied from 3 to 9 mm (mean 4.3 mm). In 16 patients the ductus resulted perfectly closed after implantation of the first double umbrella device, two patients had minimal residual aortopulmonary flow, whereas in three patients the residual shunt was significant; two of these also developed haemolysis and went to surgery, in the latter the shunt was completely abolished after implantation of a second 17-mm device 16 months later. In conclusion transcatheter closure of patent ductus arteriosus in adults is feasible, even in the presence of calcifications and/or pulmonary hypertension; taking into account the significant surgical risk, PDA umbrella closure should be considered the first choice procedure in this group of patients.     

Rastelli's procedure

In 1969, Rastelli conceived a new technique to repair of transposition of the great arteries in the presence of ventricular septal defect and severe pulmonary stenosis (TGA III), based on the redirection of ventricular outflows. An intracardiac tunnel connected the left ventricle to the aorta and an external valved conduit established continuity between the right ventricle and the pulmonary artery. TGA III and truncus arteriosus are underwent a Rastelli operation. The present report is an analysis of indication, operative technique, early and late results with this procedure. Early deaths were related to unfavourable anatomy, conduit compression and sepsis. Residual VSD and postoperative infection were the main factors contributing to the late deaths. A current Re-Rastelli operation for the problems of extracardiac valved conduit is a good result.     

Acquired aortic coarctation after surgical operation of ductus arteriosus in a preterm infant

The coarctation of the aorta can be acquired or secondary to the surgical correction of congenital anomalies of the great vessels. We report the cases of two newborns operated for patency of the ductus arteriosus (normal aortic arch and a great left-to-right-shunt). During the postoperative period the patients were asymptomatic and the echocardiogram showed no abnormalities. Later, both patients developed coarctation of the aorta. This is the first report of this condition during the neonatal period.