1/60. Congenital pulmonary venous stenosis presenting as persistent pulmonary hypertension of the newborn.Congenital pulmonary venous stenosis (CPVS) has been previously described in older infants and children, typically manifesting as failure to thrive with congestive heart failure and subsequent respiratory deterioration. We report on 2 cases of CPVS which presented during the immediate newborn period as severe persistent pulmonary hypertension of the newborn.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
2/60. Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of two affected siblings.Misalignment of pulmonary vessels with alveolar capillary dysplasia is a rare cause of persistent pulmonary hypertension of the newborn. Most of the reported cases have been sporadic. We present two consecutive affected siblings with this disorder. This is the fifth reported family occurrence of this condition. In addition to the pulmonary abnormality, one of our cases had duodenal atresia.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
3/60. Congenital dyserythropoietic anemia type I presenting as persistent pulmonary hypertension of the newborn.Congenital dyserythropoietic anemia (CDA) is a rare group of inherited bone marrow disorders characterized by anemia with ineffective erythropoiesis. We report 3 siblings from a family known to have CDA type I who presented with persistent pulmonary hypertension of the newborn (PPHN). We suggest that the diagnosis of CDA type I should be considered in any neonate with PPHN and anemia.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
4/60. Secondary infection presenting as recurrent pulmonary hypertension.Primary infection in the neonate, especially group B streptococcal infection, has long been recognized as a cause of persistent pulmonary hypertension of the newborn (PPHN), sometimes requiring treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). However, secondary nosocomial infections in the neonatal period have not been widely reported as a cause of severe recurrent pulmonary hypertension (PHTN). We now present two cases of secondary infection in the neonate leading to significant PHTN. In both cases, the infants presented with PPHN soon after birth, requiring transfer to a level 3 neonatal intensive care unit and treatment with high-frequency oscillatory ventilation and iNO. After successful resolution of the initial PPHN, including extubation to nasal cannula, both infants developed signs of severe recurrent PHTN, leading to reintubation, high-frequency oscillatory ventilation and iNO therapy, and consideration of ECMO. In both cases, blood cultures taken at the time of recurrence of PHTN returned positive, one for staphylococcus epidermidis, the other for methicillin-resistant staphylococcus aureus. These unusual cases present the possibility of severe recurrent PHTN requiring iNO or ECMO in the setting of secondary infection. We speculate that these infants, although extubated after their first episodes of PHTN, were at risk for recurrence of PHTN due to continued pulmonary vascular reactivity.- - - - - - - - - - ranking = 1.2keywords = hypertension (Clic here for more details about this article) |
5/60. Delayed-onset sensorineural hearing loss in a 3-year-old survivor of persistent pulmonary hypertension of the newborn.Persistent pulmonary hypertension of the newborn (PPHN) and its conventional medical treatment are associated with sensorineural hearing loss, yet current recommendations for regular audiological evaluations of PPHN survivors are lacking. We report a case of delayed-onset, progressive sensorineural hearing loss in a 3-year-old patient with a history of PPHN and a normal auditory brainstem evoked response at 6 weeks of age. The relatively late detection of significant sensorineural hearing loss in this otherwise healthy 3-year-old illustrates the need for audiological evaluation at regular intervals in patients with a history of PPHN.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
6/60. The use of L-arginine [correction of F-arginine] and phosphodiesterase inhibitor (dipyridamole) to wean from inhaled nitric oxide.A full-term, female neonate developed acute hypoxemic respiratory failure complicated by persistent pulmonary hypertension of the newborn (PPHN), and responded to high-frequency oscillatory ventilation (HFOV) and inhaled nitric oxide (iNO). Discontinuation of iNO was attempted three times and was followed by severe desaturation due to right-to-left shunt through the patent ductus arteriosus and patent foramen ovale. As a result of iNO dependency state and rebound pulmonary hypertension, the neonate was maintained on iNO therapy for dipyridamole alone was unsuccessful. However, successful discontinuation of iNO therapy was achieved by combination of L-arginine and dipyridamole. Exogeous NO may lead to down regulation of endogenous NO production, and further lead to rapid hydrolization of cyclic guanosine 3', 5' monophosphate (cGMP), the smooth muscle relaxant, by the enzyme phosphodiesterase. Moreover L-arginine, the precursor for the formation of endogenous NO, has been found to be deficient in neonates with PPHN, so we speculated that by inhibiting phosphodiesterase and administrating L-arginine smooth muscle relaxation occurred, and consequent weaning from iNO was achieved.- - - - - - - - - - ranking = 0.4keywords = hypertension (Clic here for more details about this article) |
7/60. Congenital neuroblastoma mimicking early onset sepsis.A newborn girl presented with symptoms of severe early onset sepsis but also with systemic hypertension (SH) at age 3 h. plasma catecholamine (CAT) levels were extremely elevated, reflecting increased release of CAT from a congenital neuroblastoma (NB). Clinical symptoms at time of admission were: prolonged capillary refill (5 s), tachycardia, tachydyspnoea, metabolic acidosis (pH 7.17, lactate 11.8 mmol/l), fever (38.4 degrees C) and SH: 90/50/65 mmHg (systolic/diastolic/mean). The infant experienced organ failure (lung, heart, liver). A retroperitoneal dumbbell tumour was detected. plasma CAT levels at age 15 h were: noradrenaline 219 nmol/l; adrenaline 13 nmol/l; and dopamine 65.3 nmol/l. SH responded to intermittent alpha-adrenergic blockage. CAT-related symptoms ceased within 1 week. The intraspinal NB was surgically removed when cord compression became symptomatic. The neurological and developmental state is normal at age 17 months. The abdominal NB regressed spontaneously. CONCLUSION: A neuroblastoma should be considered in newborn infants presenting with a shock-like condition together with systemic hypertension.- - - - - - - - - - ranking = 0.4keywords = hypertension (Clic here for more details about this article) |
8/60. Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn.The term alveolar capillary dysplasia refers to complex vascular abnormalities which have recently been identified in some infants with persistent pulmonary hypertension. We report four cases admitted to our institution for severe pulmonary hypertension unresponsive to maximal cardiorespiratory support, including high-frequency ventilation, inhaled nitric oxide and extracorporeal membrane oxygenation. The four infants died of refractory hypoxaemia. The diagnosis of alveolar capillary dysplasia was established by necropsy. We have used these cases as an opportunity for a thorough review of the literature containing comments regarding aetiology, pathophysiology, clinical presentation, associated malformations and treatment trials. CONCLUSION: alveolar capillary dysplasia should be ruled out in all newborn infants presenting severe idiopathic pulmonary hypertension associated with malformations. Open lung biopsy may prevent from using costly, invasive and probably ineffective procedures such as extracorporeal membrane oxygenation.- - - - - - - - - - ranking = 1.4keywords = hypertension (Clic here for more details about this article) |
9/60. Persistent pulmonary hypertension of the newborn due to alveolar capillary dysplasia.Three unrelated female term infants died when less than 1 month old from intractable pulmonary hypertension associated with deficient capillaries in airspace walls, anomalous small pulmonary veins in bronchiolar-arterial rays, and medial thickening in small pulmonary arteries together with peripheral muscularization. This complex vascular abnormality in the lungs has been termed alveolar capillary dysplasia and/or misalignment of lung vessels in seven previously reported cases. Each infant also showed abnormally immature parenchymal development in the lungs, as was noted in four of the seven prior cases. One had phocomelia; four of the seven prior cases had a variety of congenital anomalies. The primary pulmonary vascular anomaly is likely to be a failure of fetal lung vascularization dating from the second trimester and to be due to action of an unknown teratogen. Centroacinar veins may represent bronchial veins that do not normally develop beyond the ends of cartilaginous bronchi. Pulmonary arterial occlusive changes are interpreted as reactive to obstruction at the level of pulmonary arterioles.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
10/60. Severe neutrophilia in an infant with persistent pulmonary hypertension of the newborn.We report the case of a term male infant with severe persistent pulmonary hypertension of the newborn, who developed massive neutrophilia of unknown etiology. An extensive evaluation failed to disclose an obvious cause for his neutrophilia. A bone marrow aspirate was performed and was consistent with a leukemoid reaction. This paper reviews the neonatal leukemoid reaction and its significance.- - - - - - - - - - ranking = 1keywords = hypertension (Clic here for more details about this article) |
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