scholarly journals A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-3 ◽  
Author(s):  
Nihat Demir ◽  
Erdal Peker ◽  
İbrahim Ece ◽  
Sultan Kaba ◽  
Kemal Ağengin ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Cleft Palate ◽  
Skeletal Dysplasia ◽  
Pulmonary Hypoplasia ◽  
Persistent Pulmonary Hypertension ◽  
Term Newborn ◽  
Short Rib Polydactyly Syndrome ◽  
Renal Abnormalities ◽  
Lethal Skeletal Dysplasia

Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.

Four Patterns of Response to Inhaled Nitric Oxide for Persistent Pulmonary Hypertension of the Newborn

PEDIATRICS ◽  
1996 ◽  
Vol 98 (4) ◽  
pp. 706-713 ◽  
Author(s):  
Allan P. Goldman ◽  
Robert C. Tasker ◽  
Sheila G. Haworth ◽  
Paul E. Sigston ◽  
Duncan J. Macrae
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Time Course ◽  
Pulmonary Hypoplasia ◽  
Oxygenation Index ◽  
Pediatric Intensive Care ◽  
Inhaled Nitric Oxide ◽  
Persistent Pulmonary Hypertension ◽  
Clinical Role ◽  
Initial Trial

Objective. To determine the clinical role of inhaled nitric oxide (iNO) in the treatment of persistent pulmonary hypertension of the newborn (PPHN). Study Design. Prospective open observational clinical study. Setting. A regional cardiac and pediatric intensive care unit. Methods. Twenty-five consecutive near-term neonates (>35 weeks gestation) with severe PPHN (oxygenation index [OI]> 25) were given a trial of iNO of 20 ppm for 20 minutes. Neonates who showed a greater than 20% improvement in Pao 2 as well as a decrease in the OI to below 40 were defined as responders and continued on this therapy. Results. Four patterns of response emerged to the iNO therapy: Pattern 1 neonates (n = 2) did not respond to the initial trial of iNO—one survived. Pattern 2 neonates (n = 9) responded to the initial trial of iNO, but failed to sustain this response over 36 hours, as defined by a rise in the OI to >40. Six survived, five with extracorporeal membrane oxygenation. Pattern 3 neonates (n = 11) responded to the initial trial of iNO, sustained this response, and were successfully weaned from iNO within 5 days—all survived to discharge. Pattern 4 neonates (n = 3) responded to the initial trial of iNO, but developed a sustained dependence on iNO for 3 to 6 weeks. All three died and lung histology revealed severe pulmonary hypoplasia and dysplasia. These neonates (pattern 4) not only required iNO for a longer period of time than did the sustained responders (pattern 3), but they required significantly higher doses of iNO during their first 5 days of iNO therapy. Conclusions. Early responses to iNO may not be sustained. Neonates with pulmonary hypoplasia and dysplasia may have a decreased sensitivity and differing time course of response to iNO when compared with patients who have PPHN in fully developed lungs.

scholarly journals Inhaled Nitric Oxide at Birth Reduces Pulmonary Vascular Resistance and Improves Oxygenation in Preterm Lambs

Children ◽  
2021 ◽  
Vol 8 (5) ◽  
pp. 378
Author(s):  
Satyan Lakshminrusimha ◽  
Sylvia F. Gugino ◽  
Krishnamurthy Sekar ◽  
Stephen Wedgwood ◽  
Carmon Koenigsknecht ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Preterm Infants ◽  
Vascular Resistance ◽  
Inhaled Nitric Oxide ◽  
Persistent Pulmonary Hypertension ◽  
Inhaled No ◽  
Birth Preterm

Resuscitation with 21% O2 may not achieve target oxygenation in preterm infants and in neonates with persistent pulmonary hypertension of the newborn (PPHN). Inhaled nitric oxide (iNO) at birth can reduce pulmonary vascular resistance (PVR) and improve PaO2. We studied the effect of iNO on oxygenation and changes in PVR in preterm lambs with and without PPHN during resuscitation and stabilization at birth. Preterm lambs with and without PPHN (induced by antenatal ductal ligation) were delivered at 134 d gestation (term is 147–150 d). Lambs without PPHN were ventilated with 21% O2, titrated O2 to maintain target oxygenation or 21% O2 + iNO (20 ppm) at birth for 30 min. Preterm lambs with PPHN were ventilated with 50% O2, titrated O2 or 50% O2 + iNO. Resuscitation with 21% O2 in preterm lambs and 50%O2 in PPHN lambs did not achieve target oxygenation. Inhaled NO significantly decreased PVR in all lambs and increased PaO2 in preterm lambs ventilated with 21% O2 similar to that achieved by titrated O2 (41 ± 9% at 30 min). Inhaled NO increased PaO2 to 45 ± 13, 45 ± 20 and 76 ± 11 mmHg with 50% O2, titrated O2 up to 100% and 50% O2 + iNO, respectively, in PPHN lambs. We concluded that iNO at birth reduces PVR and FiO2 required to achieve target PaO2.

Methemoglobin and the response to inhaled nitric oxide in persistent pulmonary hypertension of the newborn

2020 ◽  
Vol 13 (2) ◽  
pp. 175-182
Author(s):  
R. Dadiz ◽  
J. Nair ◽  
C.T. D’Angio ◽  
R.M. Ryan ◽  
S. Lakshminrusimha
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Inhaled Nitric Oxide ◽  
Persistent Pulmonary Hypertension

Recombinant Human Superoxide Dismutase Enhances the Effect of Inhaled Nitric Oxide in Persistent Pulmonary Hypertension

2001 ◽  
Vol 164 (5) ◽  
pp. 834-839 ◽  
Author(s):  
ROBIN H. STEINHORN ◽  
GEORGE ALBERT ◽  
DANIEL D. SWARTZ ◽  
JAMES A. RUSSELL ◽  
CAROLYN R. LEVINE ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Superoxide Dismutase ◽  
Inhaled Nitric Oxide ◽  
Persistent Pulmonary Hypertension

The effect of inhaled nitric oxide therapy on thromboelastogram in newborns with persistent pulmonary hypertension

2014 ◽  
Vol 173 (10) ◽  
pp. 1381-1385 ◽  
Author(s):  
Sema Tanriverdi ◽  
Ozge Altun Koroglu ◽  
Ozgun Uygur ◽  
Can Balkan ◽  
Mehmet Yalaz ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Inhaled Nitric Oxide ◽  
Persistent Pulmonary Hypertension ◽  
Nitric Oxide Therapy

Persistent Pulmonary Hypertension of the Newborn: Role of Nitric Oxide

1995 ◽  
Vol 10 (6) ◽  
pp. 270-282
Author(s):  
Stella Kourembanas
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Structural Changes ◽  
Critical Role ◽  
Persistent Pulmonary Hypertension ◽  
Cell Contractility ◽  
Vasoactive Mediators ◽  
Nitric Oxide Therapy

Persistent pulmonary hypertension of the newborn (PPHN) is a common cause of respiratory failure in the full-term neonate. Molecular and cellular studies in vascular biology have revealed that endothelial-derived mediators play a critical role in the pathogenesis and treatment of PPHN. Endothelial-derived vasoconstrictors, like endothelin, may increase smooth muscle cell contractility and growth, leading to the physiologic and structural changes observed in the pulmonary arterioles of infants with this disease. On the other hand, decreased production of the endothelial-derived relaxing factor, nitric oxide, may exacerbate pulmonary vasoreactivity and lead to more severe pulmonary hypertension. Exogenous (inhaled) nitric oxide therapy reduces pulmonary vascular resistance and improves oxygenation. The safety and efficacy of this therapy in reducing the need for extracorporeal membrane oxygenation and decreasing long-term morbidity is being tested in several trials nationally and abroad. Understanding the basic mechanisms that regulate the gene expression and production of these vasoactive mediators will lead to improved preventive and therapeutic strategies for PPHN.

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