II. Epidemiology

  1. Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause)

III. Definition

  1. Partial Anomalous Pulmonary Venous Return or Connection (PAPVR)
    1. Some, but not all of the pulmonary veins return to the right atrium or a systemic vein
    2. Results in increased right sided volumes and pressures
    3. Often accompanied by a Atrial Septal Defect (ASD)
    4. Turner Syndrome increases the risk of PAPVR
  2. Total Anomalous Pulmonary Venous Return or Connection (TAPVR, TAPVC)
    1. Cyanotic Congenital Heart Defect
    2. All four pulmonary veins return to the right atrium
      1. Must be accompanied by an Atrial Septal Defect (ASD)
    3. Pulmonary venous obstruction
      1. Presents in first week of life with severe Cyanosis, pulmonary venous congestion, and poor perfusion
    4. No pulmonary venous obstruction
      1. Presents often beyond the neonatal period with mild Cyanosis and pulmonary venous congestion

IV. Differential Diagnosis

  1. Pulmonary disease
    1. TAPVR is frequently misdiagnosed initially

V. Imaging

  1. Chest XRay
    1. Ground-glass appearance of lung fields

VI. References

  1. Fujii and Lock in Cloherty (1991) Manual Neonatal Care, Little Brown, Boston, p. 262-3
  2. Soriano in Triedman (2013) Total Anomalous Pulmonary Venous Connection, UpToDate, accessed 1/9/2015

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Ontology: Scimitar Syndrome (C0036400)

Definition (NCI) A rare congenital heart disorder in which all four pulmonary veins are not connected to the left atrium and drain into the systemic veins or the right atrium instead. Infants present with cyanosis, lethargy, tachypnea, respiratory infections, and poor growth.
Definition (NCI) A rare congenital cardiopulmonary defect characterized by abnormal right-sided pulmonary venous drainage and right lung malformations.
Definition (MSH) An anomalous pulmonary venous return in which the right PULMONARY VEIN is not connected to the LEFT ATRIUM but to the INFERIOR VENA CAVA. Scimitar syndrome is named for the crescent- or Turkish sword-like shadow in the chest radiography and is often associated with hypoplasia of the right lung and right pulmonary artery, and dextroposition of the heart.
Concepts Congenital Abnormality (T019)
MSH D012587
ICD9 747.41
ICD10 Q26.2 , Q26.8
SnomedCT 204458000, 111323005, 39905002
English Syndrome, Scimitar, TAPVC - Totl anom pulm vn conn, TAPVD - Tot anom pulm vn drain, TAPVR - NOS, Total anom pulm venous connect, Total anomalous pulmonary venous return NOS, TOTAL ANOMALOUS PULMONARY VENOUS RETURN 1, APVR, SCIMITAR ANOMALY, ANOMALOUS PULMONARY VENOUS RETURN, SCIMITAR SYNDROME, Scimitar Syndrome, total anomalous pulmonary venous return, total anomalous pulmonary venous return (diagnosis), anomalous pulmonary venous return, anomalous pulmonary venous return (diagnosis), Total anomalous pulmonary venous drainage, TAPVD, Tot anom pulm ven connec, Scimitar Syndrome [Disease/Finding], tapvd, tapvr, Anomaly, Scimitar, Scimitar Anomaly, Anomalous Pulmonary Venous Return, TAPVR, Total Anomalous Pulmonary Venous Return, Pulmonary Venous Return Anomaly, TAPVR1, Total Anomalous Pulmonary Venous Return 1, Total anomalous pulmonary venous return NOS (disorder), Anomalous pulmonary venous return, Scimitar syndrome (diagnosis), Total anomalous pulmonary venous connection, Scimitar syndrome, Total anomalous pulmonary venous return, TAPVC - Total anomalous pulmonary venous connection, TAPVD - Total anomalous pulmonary venous drainage, Scimitar syndrome (disorder), Total anomalous pulmonary venous return (disorder), scimitar syndrome, connection; pulmonary veins, anomaly, total, scimitar; syndrome, syndrome; scimitar, anomaly; connection, pulmonary veins, total, v.pulmonalis; anomaly connection, total, anomaly; pulmonary, venous connection, total
Dutch totale abnormale longaderverbinding, totale abnormale longaderdrainage, anomalie; pulmonaal, veneuze verbinding, totaal, anomalie; verbinding, venae pulmonales, totaal, scimitar; syndroom, syndroom; scimitar, v.pulmonalis; anomalie verbinding, totaal, verbinding; venae pulmonales, anomalie, totaal, Abnormale verbinding van venae pulmonales, volledig, kromzwaardsyndroom, Scimitar-syndroom, Syndroom, scimitar-
French Anomalie complète du drainage veineux pulmonaire, DVPTA, RVPA total, Retour veineux pulmonaire anormal total, RVPA, RVPAT, Retour veineux pulmonaire anormal, RVPA partiel, Anomalie du retour veineux pulmonaire, Syndrome du cimeterre, Syndrome de Halasz
German vollkommen anomale Pulmonalvenenmuendung, TAPVP, Totale Fehleinmuendung der Lungenvenen, Scimitar Syndrom, Scimitar-Syndrom, Krummsäbelsyndrom, Türkensäbelsyndrom
Italian Ritorno venoso polmonare totale anomalo, Ritorno venoso anomalo polmonare totale, Connessione totalmente anomala delle vene polmonari, Sindrome della scimitarra
Portuguese Drenagem venosa pulmonar anómala total, Conexão venosa pulmonar anómala total, Síndrome de cimitarra, Síndrome de Cimitarra
Spanish DATVP, Conexión venosa pulmonar anómala total, Drenaje anómalo total de las venas pulmonares, retorno venoso pulmonar total anómalo, SAI, retorno venoso pulmonar total anómalo, SAI (trastorno), Síndrome de Drenaje Venoso Anomalo del Pulmon Derecho, conexión venosa pulmonar anómala total, retorno venoso pulmonar total anómalo (trastorno), retorno venoso pulmonar total anómalo, síndrome de la cimitarra (trastorno), síndrome de la cimitarra, Síndrome de Scimitar, Síndrome de Cimitarra
Japanese TAPVD, 総肺静脈還流異常, シミター症候群, ソウハイジョウミャクカンリュウイジョウ, シミターショウコウグン, TAPVD
Swedish Scimitarsyndrom
Czech scimitar syndrom, Scimitar syndrom, Totální anomální návrat plicních žil, Úplný anomální návrat plicních žil, Úplný anomální drenáž plicních žil
Finnish Sapelioireyhtymä
Russian TURETSKOI SABLI SINDROM, ТУРЕЦКОЙ САБЛИ СИНДРОМ
Korean 전체 폐정맥 연결이상
Polish Zespół szabli tureckiej, Zespół scimitar
Hungarian Teljes eltérő pulmonalis venás kapcsolat, TAPVD, Teljes pulmonalis eltérő venás drainage, Scimitar-syndroma
Norwegian TAPVR, Anomal lungevenedrenasje, Total anomal lungevenedrenasje, Tyrkersverdtegnet, Scimitar-syndrom

Ontology: Partial anomalous pulmonary venous connection (C0158634)

Definition (NCI) A congenital heart disorder in which one or two pulmonary veins are not connected to the left atrium and drain into the right atrium instead. It may lead to arrhythmias and pulmonary hypertension.
Concepts Congenital Abnormality (T019)
ICD9 747.42
ICD10 Q26.3
SnomedCT 204459008, 68237008
English PAPVC - Part anom pulm vn conn, PAPVD - Part anom pul vn drain, Part anom pulm vn connection, PAPVR-Part anom pul venous ret, partial anomalous pulmonary venous return, partial anomalous pulmonary venous return (diagnosis), Part anom pulm ven conn, Partial Anomalous Pulmonary Venous Return, Partial Anomalous Pulmonary Venous Connection, Partial anomalous pulmonary venous connection, Partial anomalous pulmonary venous return, PAPVC - Partial anomalous pulmonary venous connection, PAPVD - Partial anomalous pulmonary venous drainage, PAPVR - Partial anomalous pulmonary venous return, Partial anomalous pulmonary venous connection (disorder), connection; pulmonary veins, anomaly, partial, anomaly; connection, pulmonary veins, partial, v.pulmonalis; anomaly connection, partial, anomaly; pulmonary, venous connection, partial
Dutch partiële abnormale longaderverbinding, anomalie; pulmonaal, veneuze verbinding, partieel, anomalie; verbinding, venae pulmonales, partieel, v.pulmonalis; anomalie verbinding, partieel, verbinding; venae pulmonales, anomalie, partieel, Abnormale verbinding van venae pulmonales, partieel
French Retour veineux pulmonaire anormal partiel
German teilweise anomale Pulmonalvenenmuendung, Partielle Fehleinmuendung der Lungenvenen
Italian Connessione venosa polmonare parzialmente anomala
Portuguese Conexão venosa pulmonar anómala parcial
Spanish Conexión venosa pulmonar anómala parcial, retorno venoso pulmonar anómalo parcial, conexión venosa pulmonar anómala parcial (trastorno), conexión venosa pulmonar anómala parcial
Japanese 部分的肺静脈還流異常, ブブンテキハイジョウミャクカンリュウイジョウ
Czech Parciální anomální návrat plicních žil
Korean 부분 폐정맥 연결이상
Hungarian Részleges eltérő pulmonalis venás kapcsolat

Ontology: Anomalous pulmonary venous drainage (C0265916)

Concepts Congenital Abnormality (T019)
ICD10 Q26.4
SnomedCT 59631007
English APVC - Anom pulmo venous conn, APVD - Anom pulmo venous drain, Anom pulmonary venous connectn, Anomalous pulmonary venous connection, unspecified, Anomalous pulmonary venous drainage NOS, Anomalous pulmonary venous discharge, Anomalous pulmonary venous drainage, APVC - Anomalous pulmonary venous connection, APVD - Anomalous pulmonary venous drainage, Anomalous pulmonary venous connection, Anomalous pulmonary venous drainage (disorder), connection; pulmonary veins, anomaly, pulmonary; anomaly, venous connection, anomaly; communication pulmonary veins, anomaly; pulmonary, venous connection, Anomalous pulmonary venous drainage, NOS
Italian Connessione venosa polmonare anomala, Ritorno venoso polmonare anomalo NAS, Drenaggio venoso polmonare anormale, Riflusso venoso polmonare anormale
Dutch abnormale longaderdrainage NAO, abnormale longaderafscheiding, abnormale longaderdrainage, anomalie; pulmonaal, veneuze verbinding, anomalie; verbinding venae pulmonales, pulmonaal; anomalie, veneuze verbinding, verbinding; venae pulmonales, anomalie, Abnormale verbinding van venae pulmonales, niet gespecificeerd, abnormale longaderverbinding
French Anomalie du drainage veineux pulmonaire SAI, Ecoulement veineux pulmonaire anormal, Drainage veineux pulmonaire total anormal, Anomalie de la communication veineuse pulmonaire
German anomale pulmonalvenoese Drainage, anomale pulmonalvenoese Drainage NNB, anomaler pulmonalvenoeser Abfluss, Fehleinmuendung der Lungenvenen, nicht naeher bezeichnet, anomale Pulmonalvenenmuendung
Portuguese Descarga pulmonar venosa anómala, Drenagem venosa pulmonar anormal, Drenagem venosa pulmonar anormal NE, Conexão anómala das veias pulmonares
Spanish Drenaje anómalo de las venas pulmonares, Desembocadura venosa pulmonar anómala, Drenaje anómalo de las venas pulmonares NEOM, drenaje venoso pulmonar anómalo (trastorno), drenaje venoso pulmonar anómalo, Conexión anómala de las venas pulmonares
Japanese 肺静脈還流異常NOS, 肺静脈還流異常, ハイジョウミャクカンリュウイジョウ, ハイジョウミャクカンリュウイジョウNOS
Czech Anomální plicní žilní odtok, Anomální napojení plicních žil, Anomální drenáž plicních žil, Anomální drenáž plicních žil NOS
Korean 상세불명의 폐정맥 연결이상
Hungarian Pulmonalis eltérő venás váladékozás, Pulmonalis eltérő venás drainage, Pulmonalis eltérő venás drainage k.m.n., Kóros pulmonalis venás kapcsolat