Neonatology Book

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Persistent Pulmonary Hypertension of the Newborn

Aka: Persistent Pulmonary Hypertension of the Newborn, PPHN, Persistent Fetal Circulation Syndrome
  1. Epidemiology
    1. Incidence: 2 per 1000 live births
  2. Pathophysiology
    1. Underlying lung or systemic conditions (see below) prevent transition from Fetal Circulation
    2. Increased pulmonary pressures lead to right-to-left shunt and Hypoxemia
  3. Causes
    1. Meconium Aspiration Syndrome
    2. Neonatal Pneumonia
    3. Neonatal Sepsis
    4. Respiratory distress syndrome of the newborn
    5. Diaphragmatic Hernia
    6. Pulmonary hypoplasia
  4. Risk Factors
    1. Gestational Diabetes
    2. Cesarean delivery
    3. Maternal Obesity
    4. Black race
    5. Maternal SSRI use (low risk)
  5. Diagnostics
    1. Echocardiogram
  6. Signs
    1. See Neonatal Respiratory Distress
    2. Systolic Murmur
    3. Loud Second Heart Sound
    4. Hypoxia
      1. Improves with Supplemental Oxygen
  7. Management
    1. See Neonatal Respiratory Distress
    2. Consider N-CPAP or Endotracheal Intubation
    3. Consult neonatology
      1. Consider pulmonary vasodilators (Sildenafil, inhaled nitric oxide)
    4. ECMO has been used
  8. References
    1. Hermansen (2015) Am Fam Physician 92(11): 994-1002 [PubMed]

Persistent Fetal Circulation Syndrome (C0031190)

Definition (NCI) A cardiopulmonary disorder characterized by systemic arterial hypoxemia secondary to pulmonary hypertension and extrapulmonary right to left shunting across the foramen ovale and ductus arteriosus.(NICHD)
Definition (MSH) A syndrome of persistent PULMONARY HYPERTENSION in the newborn infant (INFANT, NEWBORN) without demonstrable HEART DISEASES. This neonatal condition can be caused by severe pulmonary vasoconstriction (reactive type), hypertrophy of pulmonary arterial muscle (hypertrophic type), or abnormally developed pulmonary arterioles (hypoplastic type). The newborn patient exhibits CYANOSIS and ACIDOSIS due to the persistence of fetal circulatory pattern of right-to-left shunting of blood through a patent ductus arteriosus (DUCTUS ARTERIOSUS, PATENT) and at times a patent foramen ovale (FORAMEN OVALE, PATENT).
Concepts Disease or Syndrome (T047) , Congenital Abnormality (T019)
MSH D010547
ICD9 747.83
ICD10 P29.3
SnomedCT 204507004, 35604006, 233815004, 206597007
English Circulation, Persistent Fetal, Fetal Circulation, Persistent, Hypertension, Pulmonary, of Newborn, Persistent, Persistent Fetal Circulation, Persistent Pulmonary Hypertension of Newborn, PFC - Persist fetal circulat, PPHN - Pers pulm hypert newbor, Persist pulm hypert of newborn, PFC SYNDROME, HYPERTENSION PULM OF NEWBORN PERSISTENT, CIRC PERSISTENT FETAL, PERSISTENT FETAL CIRC, PERSISTENT PULM HYPERTENSION OF NEWBORN, FETAL CIRC PERSISTENT, persistent pulmonary hypertension of the newborn, persistent fetal circulation (diagnosis), persistent fetal circulation, persistent pulmonary hypertension of newborn (diagnosis), persistent pulmonary hypertension of newborn, Persistent foetal circulation disorders, Persistent foetal circulation, Persistent fetal circ, Pulmonary Hypertension, Familial Persistent, of the Newborn, Persistent Fetal Circulation Syndrome, ALVEOLAR CAPILLARY DYSPLASIA WITH MISALIGNMENT OF PULMONARY VEINS, ALVEOLAR CAPILLARY DYSPLASIA WITH MISALIGNMENT OF PULMONARY VEINS AND OTHER CONGENITAL ANOMALIES, (Persistent) pulmonary hypertension of newborn, Persistent Fetal Circulation Syndrome [Disease/Finding], Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins And Other Congenital Anomalies, Alveolar Capillary Dysplasia With Misalignment Of Pulmonary Veins, ACDMPV, Misalignment of the Pulmonary Vessels, ACD-MPV, ACD MPV, Familial Persistent Pulmonary Hypertension of the Newborn, Newborn persistent pulmonary hypertension, Fetal circulation, Persistent Pulmonary Hypertension of the Newborn, Persistent fetal circulation syndrome, PFC - Persistent fetal circulation, PPHN - Persistent pulmonary hypertension in newborn, Persistent fetal circulation, Persistent pulmonary hypertension of the newborn, Persistent fetal circulation (disorder), Persistent fetal circulation syndrome (disorder), Persistent pulmonary hypertension of the newborn (disorder), circulation; fetal persistent, circulation; fetal, fetal; circulation, persistent, fetal; circulation, fetal; persistent circulation, persistent; circulation, fetal, persistent; fetal circulation, Persistent fetal circulation disorders, persistent foetal circulation
French Troubles liés à la persistance de la circulation foetale, Hypertension pulmonaire du nouveau-né, Hypertension artérielle pulmonaire persistante du nouveau-né, Hypertension pulmonaire persistante du nouveau-né, Circulation foetale persistante, Hypertension pulmonaire persistante des nouveau-nés, Hypertension pulmonaire persistante néonatale, Persistance de la circulation foetale
Italian Disturbi persistenti della circolazione fetale, Circolazione fetale persistente, Ipertensione polmonare persistente del neonato, Sindrome da circolazione fetale persistente
Dutch persisterende pulmonale hypertensie bij pasgeborene, circulatie; foetaal persisterend, circulatie; foetaal, foetaal; circulatie, persisterend, foetaal; circulatie, foetaal; persisterende circulatie, persisterend; circulatie, foetaal, persisterend; foetale circulatie, persisterende foetale circulatieaandoeningen, persisterende foetale circulatie, Foetale circulatie, persisterende, Foetalecirculatiesyndroom, persisterende-, Persisterende foetale circulatie, Persisterende pulmonaire hypertensie van de pasgeborene, Persisterende-foetalecirculatiesyndroom, Pulmonaire hypertensie van de pasgeborene, persisterende, Syndroom, persisterende-foetalecirculatie-
German persistierende pulmonale Hypertension beim Neugeborenen, Persistierender Fetalkreislauf, Persistierende fetale Kreislauferkrankungen, persistierender fetaler Kreislauf, Fetaler Kreislauf, persistierender, Hochdruck, pulmonaler, des Neugeborenen, persistierender, Persistierender pulmonaler Hochdruck beim Neugeborenen, Persistierendes Fetales Kreislaufsyndrom
Portuguese Hipertensão pulmonar persistente do recém-nascido, Circulação fetal persistente, Síndrome da Circulação Fetal Persistente, Afecções por persistência da circulação fetal, Persistência da circulação fetal, Circulação Fetal Persistente, Hipertensão Pulmonar Persistente do Recém-Nascido, Síndrome da Persistência do Padrão de Circulação Fetal
Spanish Hipertensión pulmonar persistente neonatal, Circulación Fetal Persistente, Síndrome de Persistencia de la Circulación Fetal, circulación fetal persistente (trastorno), circulación fetal persistente, hipertensión pulmonar persistente del recién nacido (trastorno), hipertensión pulmonar persistente del recién nacido, Circulación fetal persistente, Trastornos de circulación fetal persistente, síndrome de circulación fetal persistente (trastorno), síndrome de circulación fetal persistente, Hipertensión Pulmonar Persistente del Recién Nacido, Persistencia de la Circulación Fetal, Síndrome de Circulación Fetal Persistente
Japanese 胎児循環遺残, 持続性胎児循環障害, シンセイジセンエンセイハイコウケツアツショウ, ジゾクセイタイジジュンカンショウガイ, タイジジュンカンイザン, 胎児循環遺残症, 遺存性胎児循環症候群, 新生児持続性肺高血圧, 新生児持続性肺高血圧症, 肺高血圧症-新生児持続性, 胎児循環持続症, 胎児循環持続症候群, 新生児遷延性肺高血圧, 新生児遷延性肺高血圧症, 胎児循環残存症
Swedish Persisterande fetal cirkulation
Czech syndrom přetrvávajícího fetálního oběhu, Perzistující fetální cirkulace, Vrozené poruchy z perzistující fetální cirkulace, Persistující plicní hypertenze novorozence, syndrom perzistující fetální cirkulace, syndrom PFC, perzistující plicní hypertenze novorozence
Finnish Fetaaliverenkierto-oireyhtymä
Russian KROVOOBRASHCHENIIA PERSISTIRUIUSHCHEGO FETAL'NOGO SINDROM, FETAL'NOE KROVOOBRASHCHENIE PERSISTIRUIUSHCHEE, GIPERTENZIIA LEGOCHNAIA NOVOROZHDENNYKH PERSISTIRUIUSHCHAIA, PERSISTIRUIUSHCHAIA LEGOCHNAIA GIPERTENZIIA NOVOROZHDENNYKH, ГИПЕРТЕНЗИЯ ЛЕГОЧНАЯ НОВОРОЖДЕННЫХ ПЕРСИСТИРУЮЩАЯ, КРОВООБРАЩЕНИЯ ПЕРСИСТИРУЮЩЕГО ФЕТАЛЬНОГО СИНДРОМ, ПЕРСИСТИРУЮЩАЯ ЛЕГОЧНАЯ ГИПЕРТЕНЗИЯ НОВОРОЖДЕННЫХ, ФЕТАЛЬНОЕ КРОВООБРАЩЕНИЕ ПЕРСИСТИРУЮЩЕЕ
Korean 지속성 태아 순환
Polish Zespół przetrwałego krążenia płodowego
Hungarian Persistáló magzati keringés, Persistáló magzati keringési betegségek, Újszülött persistáló pulmonalis hypertoniája, Magzati fennmaradó keringés
Norwegian Persisterende pulmonal hypertensjon hos nyfødt, Vedvarende pulmonal hypertensjon hos nyfødt
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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