II. Epidemiology
- Accounts for 0.7 to 1.5% of Congenital Heart Disease cases (5th most common cyanotic cause)
III. Definition
- Partial Anomalous Pulmonary Venous Return or Connection (PAPVR)
- Some, but not all of the pulmonary veins return to the right atrium or a systemic vein
- Results in increased right sided volumes and pressures
- Often accompanied by a Atrial Septal Defect (ASD)
- Turner Syndrome increases the risk of PAPVR
- Total Anomalous Pulmonary Venous Return or Connection (TAPVR, TAPVC)
- Cyanotic Congenital Heart Defect
- All four pulmonary veins return to the right atrium
- Must be accompanied by an Atrial Septal Defect (ASD)
- Pulmonary venous obstruction
- Presents in first week of life with severe Cyanosis, pulmonary venous congestion, and poor perfusion
- No pulmonary venous obstruction
- Presents often beyond the neonatal period with mild Cyanosis and pulmonary venous congestion
IV. Differential Diagnosis
- Pulmonary disease
- TAPVR is frequently misdiagnosed initially
V. Imaging
-
Chest XRay
- Ground-glass appearance of lung fields
VI. References
- Fujii and Lock in Cloherty (1991) Manual Neonatal Care, Little Brown, Boston, p. 262-3
- Soriano in Triedman (2013) Total Anomalous Pulmonary Venous Connection, UpToDate, accessed 1/9/2015
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Related Studies
| 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, ｿｳﾊｲｼﾞｮｳﾐｬｸｶﾝﾘｭｳｲｼﾞｮｳ, ｼﾐﾀｰｼｮｳｺｳｸﾞﾝ, 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, ﾌﾞﾌﾞﾝﾃｷﾊｲｼﾞｮｳﾐｬｸｶﾝﾘｭｳｲｼﾞｮｳ |
| 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, ﾊｲｼﾞｮｳﾐｬｸｶﾝﾘｭｳｲｼﾞｮｳ, ﾊｲｼﾞｮｳﾐｬｸｶﾝﾘｭｳｲｼﾞｮｳ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 |
