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This article describes a technique which allows the patent ductus arteriosus (PDA) to be closed through a minithoracotomy. It combines a simple method of inducing atelectasis with Ligaclip closure of the PDA through a subaxillary incision. It was used successfully in 11 children and adolescents.
We report a neonate with distal bilateral ductus arteriosus origin of nonconfluent pulmonary arteries which was only diagnosed accurately by 2-D echo Doppler color flow mapping in the context of situs ambiguous, univentricular A-V connection and pulmonary atresia with normal systemic/pulmonary venous drainage. The color flow appearance of this singular vascular anatomy was enhanced after prostaglandin administration, which, allowed us to see an image resembling a "moustache morphology". At 36 months of age, and after a successful bilateral 4 mm modified Blalock Taussig shunt implantation by sequential approach, the patient is waiting for the next surgical step which will include a restoration of the confluence of the pulmonary arteries with bidirectional Glenn operation. Brief embryological and differential diagnosis comments are discussed.
Diagnosis of an isolated patent ductus arteriosus (PDA) is usually straightforward. If at later age it is associated with pulmonary hypertension, however, the symptoms may be variable and more difficult to assess. In the age group primarily discussed here, pulmonary hypertension is frequently present, so diagnosis cannot be based on physical findings alone. Echocardiography and Doppler echocardiography are essential diagnostic procedures; however, cardiac catheterization and angiography may still be needed especially in cases with associated heart defects. At present, surgical closure of the PDA is the therapy of choice in infants. In preterm newborns, an attempt by medical treatment is indicated and often promising. Unfortunately, there are no interventional techniques available at present for duct closure in this age group, whereas in some centers catheter closure of a PDA is successfully employed in older children.
Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.
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We report a case of prenatal diagnosis of premature closure of the ductus arteriosus following maternal diclofenac therapy at 35 weeks of gestation. Fetal echocardiography at 37 weeks of gestation revealed a dilated right ventricle with moderate tricuspid regurgitation, pulmonary insufficiency and complete closure of the ductus arteriosus. Immediate Cesarean section resulted in an excellent neonatal outcome. Whereas the effect of indomethacin on prenatal ductal constriction is well known, widely prescribed non-steroidal anti-inflammatory drugs such as diclofenac can have an equally deleterious effect and are best avoided in the third trimester of pregnancy.
Programmed proliferative degeneration of the human fetal ductus arteriosus (DA) in preparation for its definite postnatal closure has a large developmental variability and is controlled by several signaling pathways, most prominently by prostaglandin (PG) metabolism. Numerous studies in various mammalian species have shown interspecies and developmental differences in ductal protein expression of cyclooxygenase (COX) isoforms and PG E receptor subtypes (EP1-4). We examined COX1, COX2, and EP4 receptor protein expression immunohistochemically in 57 human fetal autopsy DA specimens of 11-38 wk of gestation. According to their histologic maturity, specimens were classified into four stages using a newly designed maturity score that showed that histologic maturity of the DA was not closely related to gestational age. COX1 expression was found in all DA regions and rose steadily during development. COX2 staining remained weak throughout gestation. EP4 receptor staining increased moderately during gestation and was limited to the intima and media. In conclusion, histologic maturity classification helps to address developmentally regulated processes in the fetal DA. Concerning prostaglandin metabolism our findings are in line with animal studies, which assigned COX1 the predominant role in the DA throughout gestation. EP4 receptor presumably plays a key role for active patency of the human DA in the third trimester.
To assess the influence of ductal size on the results of transcatheter closure of patent ductus arteriosus (PDA) with coils, 154 consecutive patients were studied prospectively. Ductal size was defined as the narrowest diameter of ductus measured on aortography. All patients were divided into 5 groups according to ductal size: < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm. The occlusion of PDA with coils was performed through a transarterial approach. The results were evaluated by angiography at 10 minutes and by color Doppler echocardiography at 1 day, 2 days, 1 week, 1 month, 3 months, 6 months, and 12 months after the procedure. The immediate occlusion rates for ductal sizes < 1 mm, 1-1.9 mm, 2-2.9 mm, 3-3.9 mm, and > or = 4 mm were 89.7%. 75.4%, 51.4%, 30.8%, and 40%, respectively; whereas the occlusion rates at 12-months follow-up were 100%, 98.5%, 97.3%, 69.2%, and 80%, respectively. There were no significant differences in occlusion rate at 12-months follow-up among the groups with ductal sizes < 3 mm or among the groups with ductal sizes > or = 3 mm. The occlusion rate for ductal size < 3 mm at each follow-up time was significantly higher than that for ductal size > or = 3 mm (10 minutes: 71.8% vs 34.8%. P = 0.001; 12-months: 98.5% vs 73.9%, P < 0.001). The occlusion rate of residual shuntings at 12-months follow-up for ductal size < 3 mm was also significantly higher than that for ductal size > or = 3 mm (94.6% vs 60%, P = 0.007). The results of the present study demonstrate that ductal size < 3 mm had a higher occlusion rate than that for a size > or = 3 mm. PDA with a size > or = 3 mm may need other treatment strategies or other devices to achieve better results.
Although the role of PGE2 in maintaining ductus arteriosus (DA) patency is well established, the specific PGE2 receptor subtype(s) (EP) involved have not been clearly identified. We used late gestation fetal and neonatal lambs to study developmental regulation of EP receptors. In the fetal DA, radioligand binding and RT-PCR assays virtually failed to detect EP1 but detected EP2, EP3D, and EP4 receptors in equivalent proportions. In the newborn lamb, DA total density was one-third of that found in the fetus and only EP2 was detected. Stimulation of EP2 and EP4 increased cAMP formation and was associated with DA relaxation. Though stimulation of EP3 inhibited cAMP formation, it surprisingly relaxed the fetal DA both in vitro and in vivo. This EP3-induced relaxation was specifically diminished by the ATP-sensitive K(+) (K(ATP)) channel blocker glibenclamide. In conclusion, PGE2 dilates the late gestation fetal DA through pathways that involve either cAMP (EP2 and EP4) or K(ATP) channels (EP3). The loss of EP3 and EP4 receptors in the newborn DA is consistent with its decreased responsiveness to PGE2.
OBJECTIVE: We reviewed the experience of 2 centers performing surgical ligation of patent ductus arteriosus in preterm neonates to identify whether the choice of surgical technique--suture ligation or clip application--affected outcome. METHODS: Between 2000 and 2005, 67 newborn infants had open surgical closure of patent ductus arteriosus: 33 by suture ligation and 34 by clip application. The groups were similar in age and sex. RESULTS: The average length of the procedure was 55.8 +/- 13.7 minutes for suture ligation and 30.8 +/- 8.7 minutes for clip application (P < .05). Six neonates had intraoperative bleeding in the suture ligation group. Four patients had significant postoperative complications in the suture ligation group, compared with 2 in the clip application group. CONCLUSIONS: This study demonstrates that clip application results in a significant reduction in the operative time and, possibly, in less morbidity.
INTRODUCTION: Persistent patent ductus arteriosus (PDA) often produces hemodynamic and respiratory derangement necessitating use of inotropic drugs and escalating ventilatory support in premature infants. When medical therapy fails, surgical ligation is indicated. Because of the risks of transferring unstable neonates to the operating room, ductal ligation is routinely performed at the neonatal intensive care unit (NICU) bedside. Some patients, however, require transfer from hospitals without pediatric cardiac surgical teams. In an attempt to eliminate the risks associated with transfer, a surgical team from our institution offered to perform duct ligation in the NICUs of referring institutions. This experienced team consisted of a pediatric cardiac attending anesthesiologist and certified registered nurse anesthetist, cardiac operating room nurses, an attending cardiothoracic surgeon, and a cardiothoracic surgery fellow. We retrospectively reviewed our experience. METHODS: After approval from the Committee for the Protection of Human Subjects, the charts of premature neonates who underwent PDA ligation in the NICU at the Children's Hospital of Philadelphia NICU or in a network NICU between January 1996 and April 2002 were reviewed. Data abstracted included institution, gender, gestational age, birth weight, weight at surgery, and number of courses of indomethacin. Mean arterial blood pressure and use of inotropic drugs and ventilatory parameters (fraction of inspired oxygen, peak inspiratory pressure) were recorded at the time of surgery and 96 hours postoperatively. Perioperative complications were recorded. RESULTS: Seventy-two patients met the criteria for inclusion. PDA ligation was performed in the Children's Hospital of Philadelphia NICU in 38 of 72 patients, 53% (group 1). The remainder, 34 of 72 (47%) underwent PDA ligation in the NICU at 1 of 6 referring institutions (group 2). There were no significant differences between groups with respect to demographics, number of courses of indomethacin, or use of inotropic drugs or ventilatory support. The incidence of perioperative complications did not differ between groups: 3 in group 1 (bleeding, chylothorax, and pleural effusion) and 3 in group 2 (pneumothorax [3]). There were no anesthetic-related complications. Seven patients died (4 in group 1 and 3 in group 2), none within 96 hours of surgery and none secondary to the procedure. DISCUSSION: The data demonstrate that an experienced team can perform PDA ligation safely in NICUs of hospitals without on-site pediatric cardiac surgical capabilities in critically ill neonates without incurring the risks inherent in patient transport. Most importantly, patient care is continued by the neonatology team most familiar with the infant's medical and social history, and the patient's family is minimally inconvenienced.
We present the results and follow up of ductus arteriosus closure with the Rashkind device. Sixty-six devices were implanted in 63 patients, 41 women and 22 men, with a mean age of 8.2 years. We determined the diameter of the duct, the presence or absence of immediate residual leak at 3 months, 6 months, 1 year, and every year of follow-up by ecocardiography. The hemodynamic determinations showed: type A morphology in 49, type C in 7 and type E in 7; with a mean diameter of 4.2 +/- 1.4 mm (range of 2.3 to 8.7 mm) and a Qp/Qs 2.2 +/- 1.5 (range 0.7 to 8.6). Forty-five 17 mm devices and 18 of 12 mm were used. The incidence of immediate residual leak was 65% (n-41); at 24 hrs. Post-procedure was 31.7% (n-20) and at one year 7.9% (n-5). Three patients with persistent leak had implantation of a second Rashkind device and one more patient an endovascular coil, the other patient is waiting for a second occlusor. Minor complications occurred in 15.8% (n-10). Results show an occlusion rate at 24 hrs of 68% and at one year of 92%; no patient presented migration of the device, hemolysis or obstruction of the pulmonary artery branches. We consider that occlusion of the ductus arteriosus with the Rashkind device is a safe and effective option for the treatment of this pathology.
Precise delineation of the size and shape of the ductus arteriosus may help in catheter closure. The use of Intravascular Ultra Sound (IVUS) to study the diameter of patent ductus arteriosus (PDA) has not been reported. We prospectively evaluated the use of IVUS and compared it to angiography in determining the size and shape of PDA and to assess the degree of coil protrusion into the descending aorta and the clinical relevance after coil closure. Twenty-six patients (17 female, 9 male) underwent transcatheter closure of PDA using single or multiple Gianturco coils. The median age of the patients was 1.7 yr (range, 2 monthsÐ34 yr) and the median weight was 10.9 kg (range 4.2Ð80 kg). A good quality IVUS image was obtained in all patients using 3.8 Fr, 30 MHz catheters. The median PDA diameter measured by angiography was 2.8 mm (range 1.6Ð5.7 mm) and by IVUS was 3.2 mm (range 1.7Ð5.7). There was excellent agreement between IVUS and angiography (D IVUS = 0.25+0.97*D angio, r = 0.90, (D: diameter in mm). After closure, coils could be seen protruding into the aorta with a median percent coil protrusion of 17.3% (range 0.0Ð47%). Only one patient had the coil protruding into more than 1/2 the aorta. This coil was retrieved and another coil was placed successfully. On follow-up at a median interval of 2.4 yr. (range 1.1Ð3.2 yr), there has been no clinical evidence for thromboembolic events. We conclude that IVUS use to assess the size of PDA is accurate. On mid-term follow-up, coil protrusion into the aorta seems to be benign. Further follow-up is needed.
A case is presented of vascular ring caused by right aortic arch with mirror-image branching and left ductus arteriosus. In this case, the descending aorta was located right of the tracheoesophagus and the left ductus arteriosus connected to the descending aorta far below the arch, producing compression of the esophagus only. Through median sternotomy, the ligation and division of the ductus was performed with concomitant repair of ventricular septal defect.
Three recent cases of surgical closure of ductus arteriosus in very low birth weight infants (less than 1 000 g) are reported. The indications of both surgical and pharmacological closure in very low birth weight premature infants are discussed. The necessity of an early closure of a patent ductus arteriosus is now admitted in the very premature infant, but the use of indomethacin in the first days of life is often impossible because of its side-effects. The use of adapted non-invasive monitoring devices during surgery allows a better control of anaesthesia.
An experimental model using rats is described, in which a state of copper deficiency was produced in pregnant dams and their offspring. In 100% of the neonates from the copper-deficient group (10/10), 20% (2/10) from the control group, and 40% (4/10) from the pair-fed group, the ductus arteriosus was found to remain patent. These figures were highly significant in statistical terms (X2 test; p less than 0.0009). The possible physiopathology of the persistence of the ductus arteriosus in copper-deficient neonates is discussed.
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