Aorta, Thoracic ; abnormalities ; Child, Preschool ; Ductus Arteriosus, Patent ; complications ; Female ; Humans ; Pulmonary Artery ; abnormalities ; Subclavian Artery ; abnormalities

Aortic Coarctation ; complications ; Ductus Arteriosus, Patent ; complications ; Humans ; Infant ; Pulmonary Artery ; abnormalities

Isolated absence of the ductus arteriosus is extremely rare condition although the ductus arteriosus may be hypoplastic or aplastic in association with other aortic arch anomalies. Authors described a case of isolated agenesis of the ductus arteriosus documented by postmortem examination of a newborn infant who died of pneumonia following operation for a large omphalocele. The heart showed ventricular septal defect. However, no other cardiovascular anomalies were associated in this case. There were three vessels that were taking off from the aorta consisted of the right brachiocephalic artery, left common carotid-artery and left subclavian artery. The anteriorly located pulmonary artery was divided into the right and left pulmonary arteries. There was no connection of vessel between the pulmonary artery and the aorta.
Abnormalities, Multiple/embryology/pathology ; Ductus Arteriosus/*abnormalities ; Heart Defects, Congenital/embryology/pathology ; Hernia, Umbilical/complications ; Humans ; Infant, Newborn ; Male

Ductus Arteriosus ; abnormalities ; Fatal Outcome ; Female ; Heart Septal Defects, Atrial ; etiology ; Humans ; Infant, Newborn ; Rubella Syndrome, Congenital ; complications

OBJECTIVEThe development of pulmonary vascular bed is strongly flow-dependent. Abnormal pulmonary blood flow leads to pulmonary pathological changes. This study aimed to observe the pathological changes of small pulmonary arteries and alveoli in complex congenital heart defect with diminished pulmonary blood flow but without aortopulmonary collateral artery (APCA) and patent ductus arteriosus (PDA) in infants and young children.

METHODSAutopsy pulmonary specimens obtained from 5 children who died of non-cardiovascular diseases were used as the control group (age: 4-18 months). Fifty-six children (age: 4-36 months) with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA served as the study group, including 34 cases of tetralogy of Fallot, 7 cases of double outlet right ventricle with pulmonary stenosis, 9 cases of single ventricle with pulmonary stenosis, 4 cases of tricuspid atresia with pulmonary stenosis and 2 cases of complete atrioventricular septal defect with pulmonary stenosis. Pulmonary specimen sections were stained by hematoxylin-eosin and Weigert-Van Gieson. Percentage of media thickness (MT%), percentage of media section area (MS%), number of small arterial per square centimeter (APSC), mean alveolar number (MAN), mean linear intercept (MLI), proportion of parenchyma area in total area (PPA%) and alveolar to small arterial ratio per unit area (AAR) were measured by morphologic quantitative analysis.

RESULTSMT% (10.93+/-2.87% vs 15.08+/-2.51%), MS% (18.97+/-5.56% vs 25.04+/-3.87%) and APSC (202.43+/-67.45 vs 441.69+/-65.29) decreased significantly in the study group compared with the control group (P<0.01). The internal diameter of small pulmonary artery (80.26+/-21.57 microm vs 58.53+/-10.29 microm; P<0.05), AAR (46.59+/-14.43 vs 34.46+/-4.98; P<0.01) and MLI (144.98+/-44.87 microm vs 108.39+/-20.76 microm; P<0.05) increased significantly compared with the control group.

CONCLUSIONSThe media of small pulmonary arteries becomes thinner, the lumen of small pulmonary arteries becomes larger, and the number of small arterial per square centimeter and the mean alveolar number are reduced in infants and young children with complex congenital heart defect with diminished pulmonary blood flow but without APCA and PDA.

Aorta ; abnormalities ; Child, Preschool ; Collateral Circulation ; Ductus Arteriosus, Patent ; pathology ; Female ; Heart Defects, Congenital ; pathology ; Humans ; Infant ; Lung ; pathology ; Male ; Pulmonary Artery ; abnormalities ; Pulmonary Circulation

Abnormalities, Multiple ; surgery ; Adult ; Ductus Arteriosus, Patent ; surgery ; Heart Defects, Congenital ; surgery ; Heart Septal Defects, Ventricular ; surgery ; Humans ; Levocardia ; surgery ; Male ; Vena Cava, Inferior ; abnormalities

The DiGeorge syndrome is a rare congenital abnormality consisting of aplasia or hypoplasia of the thymus and parathyroid glands resulting from malformation of the third and fourth pharyngeal pouches. This syndrome usually includes congenital cardiac anomalies and abnormal facial features. We experienced a case of congenital cardiac anomaly associated with DiGeorge syndrome. The patient was 1 month old boy weighing 3.5 kg. The congenital cardiac anomalies included ventricular septal defect, atrial septal defect, coactation of aorta, and patent ductus arteriosus. We performed one-stage operation with two separate incisions for these cardiac anomalies. Postoperative course was uneventful and the patient at 6 months of follow up is doing well.
Aorta ; Congenital Abnormalities ; DiGeorge Syndrome* ; Ductus Arteriosus, Patent ; Follow-Up Studies ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Humans ; Infant, Newborn ; Male ; Parathyroid Glands ; Thymus Gland

PHACE association is a rare neurocutaneous condition in which facial hemangiomas associate with a spectrum of posterior fossa malformations, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. We reported a case of PHACE association in a premature infant showing facial, intracranial, and oropharyngeal hemangiomas with evidence of the Dandy-Walker variant and complicated cardiovascular anomalies, including a right-sided aortic arch and an atypical patent ductus arteriosus arising from a tortuous left subclavian artery. To our knowledge, intracranial hemangiomas are rare in PHACE association, and a concomitant oropharyngeal hemangioma has not been previously reported in the PHACE association literature. In infants presenting with large, plaque-like facial hemangiomas, it is important to conduct active cardiovascular and neurological evaluations. Special attention should be given to the laryngoscopic examination to search for additional hemangiomas in the airway.
Aorta, Thoracic ; Aortic Coarctation ; Ductus Arteriosus, Patent ; Eye ; Eye Abnormalities ; Hemangioma ; Hemangioma, Cavernous, Central Nervous System ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Neurocutaneous Syndromes ; Subclavian Artery

Mixed gonadal dysgenesis (MGD) with 45X/XY karyotype and patent ductus arteriosus (PDA) is a rare congenital abnormality. A thirty eight month-old male patient was admitted to our urology department for evaluation of ambiguous genitalia. On physical examination, a ptosis, a wide neck with a low posterior hairline, small phallus, testis in the right labioscrotal fold, urethral opening on the penoscrotal junction, and typical findings of patent ductus arteriosus were present. The chromosome karyotype of the patient was 45X/XY. On the laparotomy there were infantile fallopian tubes, ovary and a immature testis on the left. The treatment of the patien1 included gender identity. excision of mullerian structure, left gonadectomy, urethroplasty, biopsy of right testis and double ligation of PDA. We report a case of mixed gonadal dysgenesis with 45X/XY karyotype and patent ductus arteriosus.
Biopsy ; Congenital Abnormalities ; Disorders of Sex Development ; Ductus Arteriosus, Patent* ; Fallopian Tubes ; Female ; Gender Identity ; Gonadal Dysgenesis, Mixed* ; Humans ; Karyotype* ; Laparotomy ; Ligation ; Male ; Neck ; Ovary ; Physical Examination ; Testis ; Urology

OBJECTIVEIn order to provide pathologic reference for therapeutic rationale, the pathological changes of the pulmonary vasculature in patients with pulmonary atresia with ventricular defect and patent ductus arteriosus were observed by contrast with normal control group.

METHODSLung biopsies were taken in the operation in 10 children suffered from pulmonary atresia with ventricular septal defect associated with patent ductus arteriosus (PA group). Autopsy specimens were obtained from 10 children died of non-cardiovascular diseases as normal control group. The tissue was fixed with buffered formalin, routinely prepared by impregnated in wax. Sections were stained by hematoxylin-eosin, Weigert's elastic stain counter-stained by van Gieoson's method. Seven parameters were obtained including percentage of media thickness (MT%), percentage of media section area (MS%), numbers of vascular per square centimeter (VPSC), mean alveolar number (MAN), mean linear intercept (MLI), proportion of parenchyma area in total area (PPA), and alveolar/vascular ratio per unit area (AVR) by a computer image processor by quantitative analysis.

RESULTSThere were significant difference between the two groups in MAN, VPSC, and AVR (P < 0.05). VPSC was significantly lower in PA group than in control group (P < 0.01). Other parameters had no significant difference. The mean alveolar diameter had an increased trend in PA group, although there was no significant difference. MS% of nearly 50% patients was closed to the normal value in PA group. The shape of pulmonary arteriole was irregular. There were few muscular arteries in a field of vision.

CONCLUSIONSThe density of muscular arteries decreases in patients with pulmonary atresia with ventricular septal defect and patent ductus arteriosus, but percentage of media thickness and percentage of media section area of pulmonary arterioles are close to the normal value. Diminished flow in pulmonary circulation has a significant effect on numbers of pulmonary arterioles per square centimeter that impact the selection of surgical method and the effect of operation because of the reduction pulmonary arterioles. The decrease of mean alveolar number results in compensatory enlargement of alveolar diameter. The impaired lung development is a major cause of abnormal lung function. Feasible and earlier operation, which can increase pulmonary flow and promote development of pulmonary vasculature will be helpful to restore lung function.

Abnormalities, Multiple ; pathology ; Child, Preschool ; Ductus Arteriosus, Patent ; pathology ; Female ; Heart Septal Defects, Ventricular ; pathology ; Humans ; Infant ; Male ; Pulmonary Artery ; pathology ; Pulmonary Atresia ; pathology