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PP-57. Hypoplastic right heart syndrome (HRHS)
Abstracts
under general anesthesia in the operating room. The distance to these centers was 5–50 (23.4 ± 20.29) kilometers and the travel time was 2.5–28 (12.57 ± 11.44) minutes. The mean gestational age was 27 weeks and the mean weight at the time of the procedure was 650–1850 (1248.6 ± 398.3) grams. 17 (85%) patients had mechanic ventilation support at the time of transport. In 18 (90%) babies medical treatment had been applied. They were operated between ages of 2 and 71 days (mean 41 days). All patients were transferred back to their own centers in transport incubator in hemodynamically stable status under control of a physician.
S41
surgical team. The relationship between the anomaly and in-vitro fertilization should be considered. doi:10.1016/j.earlhumdev.2010.09.110
PP-58. Persistent pulmonary hypertension in the neonate Nijole Drazdiene, Ramune Vankeviciene Vilnius University Children Hospital, Lithuania
Results
Aim
Mean procedural time was 54 min. There was no surgical or early mortality. All patients were transferred back to their centers within 24 h, postoperatively. In the first two weeks following surgery,13 (65%) babies were successfully weaned from mechanic ventilation support. 3 (15%) babies died in the late term due to sepsis and intracranial bleeding.
Persistent pulmonary arterial hypertension (PPAH) of the newborn is a major clinical problem in the neonatal intensive care unit. PPAH occurs in parenchymal lung disease (respiratory distress syndrome and meconium aspiration syndrome), pulmonary hypoplasia (congenital diaphragmatic hernia) or idiopathic. Primary pulmonary hypertension is a very rare condition with a bad prognosis. The aim was to assess the incidence of persistent and idiopathic pulmonary hypertension in the neonates.
Conclusions As the technology of patient transport vehicles and instruments developed and the skills of the medical personnel improved, the cardiac centers had the opportunity to safely operate low-birth weight babies followed in other centers without cardiac surgery clinics. We did not observe any morbidity and mortality related to transportation and cardiac procedure in 20 patients who were uneventfully transferred back in 24 h.
Materials and methods We studied the incidence of persistent pulmonary hypertension in the neonatal intensive care unit during the period 2002–2009 years in Vilnius University Children Hospital. Results
doi:10.1016/j.earlhumdev.2010.09.109
PP-57. Hypoplastic right heart syndrome (HRHS) Hajrije Ismaili, Lindita Kryeziu, Burbuqe Skenderi Mustafa, Ramush Bejiqi Gynecology Clinic NICU, Prishtina, Kosovo Aim The aim of our work is to present one of our newborn babies with hypoplastic right heart syndrome (HRHS), tricuspid atresia. HRHS is a congenital heart defect in which the right ventricle of the heart fails to grow and develop appropriately. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant. Case presentation A male preterm baby, 36 week gestational age, after assisted conception, delivered by cesarean section with a birth weight of 2300 g; length 44 cm, was admitted to hospital with generalized cyanosis. Apgar score at 1 and 5 min was 6 and 8 respectively. Vital signs were as follows: respiratory rate: 50/min; heart rate: 155/min; SpO2: 70–75%, with or without O2HOOD. Heart: in auscultation: rhythmic heart beat, clear tones, systolic murmur in whole precordium, the intensity III/6*. The echocardiography showed hypoplasia of right ventricle; tricuspid atresia; pulmonary artery stenosis, patent ductus arteriosus and foramen ovale. Prostaglandin treatment was started and the baby was referred to a level 3 hospital.
During this period 1989 newborns were treated in neonatal intensive care unit. Severe perinatal hypoxia with meconium aspiration syndrome, congenital pneumonia or congenital diaphragmatic hernia was diagnosed in 127 (6.4%) term newborns. 56 (44.1%) of them was meconium aspiration syndrome, 45 (35.4%) — respiratory distress syndrome and 26 (20.5%) — congenital diaphragmatic hernia. Persistent pulmonary hypertension was diagnosed in 82 (64.6%) of these newborns. The initial treatment includes correction of hypothermia, acidosis, hypoglycemia, hypocalcemia, anemia and hypovolemia. Most of these newborns were treated with highfrequency oscillatory ventilation, and cardiac function was optimized with inotropic drugs (dobutamine, dopamine and sometimes milrinone). 106 (83.5%) newborns survived. Idiopathic (primary) pulmonary hypertension was diagnosed in one newborn on 17 days of life. This neonate was initially treated with calcium-channelantagonist (diltiazem) for two months, later — with sildenafil. Today this child is 10 months old, pulmonary hypertension is high (about 80 mm Hg), and he is waiting for treatment with bosentan. Conclusions Persistent pulmonary hypertension is a severe disease in neonatal period, but most of newborns survived (83.5%). Idiopathic pulmonary hypertension is a very rare disease with a bad prognosis. doi:10.1016/j.earlhumdev.2010.09.111
Conclusion
PP-59. Therapeutic combination of sildenafil and inhaled iloprost in a preterm neonate with pulmonary hypertension
This rare anomaly requires prenatal diagnosis since it needs immediate and emergency treatment. Pregnant women whose pregnancy complicated with this anomaly should be referred to a level 3 hospital with pediatric cardiology and pediatric cardiothoracic
Petek Kayırana, Berkan Gürakana, Sinan Mahir Kayıranb, Nilüfer Öztürkc, Aygün Dindard a American Hospital, Neonatal Intensive Care Unit, Turkey b American Hospital, Department of Pediatrics, Turkey
under general anesthesia in the operating room. The distance to these centers was 5–50 (23.4 ± 20.29) kilometers and the travel time was 2.5–28 (12.57 ± 11.44) minutes. The mean gestational age was 27 weeks and the mean weight at the time of the procedure was 650–1850 (1248.6 ± 398.3) grams. 17 (85%) patients had mechanic ventilation support at the time of transport. In 18 (90%) babies medical treatment had been applied. They were operated between ages of 2 and 71 days (mean 41 days). All patients were transferred back to their own centers in transport incubator in hemodynamically stable status under control of a physician.
S41
surgical team. The relationship between the anomaly and in-vitro fertilization should be considered. doi:10.1016/j.earlhumdev.2010.09.110
PP-58. Persistent pulmonary hypertension in the neonate Nijole Drazdiene, Ramune Vankeviciene Vilnius University Children Hospital, Lithuania
Results
Aim
Mean procedural time was 54 min. There was no surgical or early mortality. All patients were transferred back to their centers within 24 h, postoperatively. In the first two weeks following surgery,13 (65%) babies were successfully weaned from mechanic ventilation support. 3 (15%) babies died in the late term due to sepsis and intracranial bleeding.
Persistent pulmonary arterial hypertension (PPAH) of the newborn is a major clinical problem in the neonatal intensive care unit. PPAH occurs in parenchymal lung disease (respiratory distress syndrome and meconium aspiration syndrome), pulmonary hypoplasia (congenital diaphragmatic hernia) or idiopathic. Primary pulmonary hypertension is a very rare condition with a bad prognosis. The aim was to assess the incidence of persistent and idiopathic pulmonary hypertension in the neonates.
Conclusions As the technology of patient transport vehicles and instruments developed and the skills of the medical personnel improved, the cardiac centers had the opportunity to safely operate low-birth weight babies followed in other centers without cardiac surgery clinics. We did not observe any morbidity and mortality related to transportation and cardiac procedure in 20 patients who were uneventfully transferred back in 24 h.
Materials and methods We studied the incidence of persistent pulmonary hypertension in the neonatal intensive care unit during the period 2002–2009 years in Vilnius University Children Hospital. Results
doi:10.1016/j.earlhumdev.2010.09.109
PP-57. Hypoplastic right heart syndrome (HRHS) Hajrije Ismaili, Lindita Kryeziu, Burbuqe Skenderi Mustafa, Ramush Bejiqi Gynecology Clinic NICU, Prishtina, Kosovo Aim The aim of our work is to present one of our newborn babies with hypoplastic right heart syndrome (HRHS), tricuspid atresia. HRHS is a congenital heart defect in which the right ventricle of the heart fails to grow and develop appropriately. This defect causes inadequate blood flow to the lungs and thus, a blue or cyanotic infant. Case presentation A male preterm baby, 36 week gestational age, after assisted conception, delivered by cesarean section with a birth weight of 2300 g; length 44 cm, was admitted to hospital with generalized cyanosis. Apgar score at 1 and 5 min was 6 and 8 respectively. Vital signs were as follows: respiratory rate: 50/min; heart rate: 155/min; SpO2: 70–75%, with or without O2HOOD. Heart: in auscultation: rhythmic heart beat, clear tones, systolic murmur in whole precordium, the intensity III/6*. The echocardiography showed hypoplasia of right ventricle; tricuspid atresia; pulmonary artery stenosis, patent ductus arteriosus and foramen ovale. Prostaglandin treatment was started and the baby was referred to a level 3 hospital.
During this period 1989 newborns were treated in neonatal intensive care unit. Severe perinatal hypoxia with meconium aspiration syndrome, congenital pneumonia or congenital diaphragmatic hernia was diagnosed in 127 (6.4%) term newborns. 56 (44.1%) of them was meconium aspiration syndrome, 45 (35.4%) — respiratory distress syndrome and 26 (20.5%) — congenital diaphragmatic hernia. Persistent pulmonary hypertension was diagnosed in 82 (64.6%) of these newborns. The initial treatment includes correction of hypothermia, acidosis, hypoglycemia, hypocalcemia, anemia and hypovolemia. Most of these newborns were treated with highfrequency oscillatory ventilation, and cardiac function was optimized with inotropic drugs (dobutamine, dopamine and sometimes milrinone). 106 (83.5%) newborns survived. Idiopathic (primary) pulmonary hypertension was diagnosed in one newborn on 17 days of life. This neonate was initially treated with calcium-channelantagonist (diltiazem) for two months, later — with sildenafil. Today this child is 10 months old, pulmonary hypertension is high (about 80 mm Hg), and he is waiting for treatment with bosentan. Conclusions Persistent pulmonary hypertension is a severe disease in neonatal period, but most of newborns survived (83.5%). Idiopathic pulmonary hypertension is a very rare disease with a bad prognosis. doi:10.1016/j.earlhumdev.2010.09.111
Conclusion
PP-59. Therapeutic combination of sildenafil and inhaled iloprost in a preterm neonate with pulmonary hypertension
This rare anomaly requires prenatal diagnosis since it needs immediate and emergency treatment. Pregnant women whose pregnancy complicated with this anomaly should be referred to a level 3 hospital with pediatric cardiology and pediatric cardiothoracic
Petek Kayırana, Berkan Gürakana, Sinan Mahir Kayıranb, Nilüfer Öztürkc, Aygün Dindard a American Hospital, Neonatal Intensive Care Unit, Turkey b American Hospital, Department of Pediatrics, Turkey