sindrom scimitar
TRANSCRIPT
![Page 1: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/1.jpg)
MALFORMATII VENOASE
PULMONARE
![Page 2: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/2.jpg)
DEFINITIE
Malformatii ale venelor pulmonare care se varsa in alt loc decat in atriul stang
pot fi - totale (TAPVR)-toate cele 4 vene pulmonare
- partiale (PAPVR)-1-3 vene pumonare
TAPVR reprezinta 2% din totalul malformatiilor cardiace congenitale
![Page 3: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/3.jpg)
CLASIFICARE (Darling) Tip 1: supracardiaca (50% din cazuri)- cele 4 vene
pulmonare conflueaza intr-un colector venos orizontal situat in spatele atriului stang, care se continua cu o portiune verticala care se varsa in VCS ( cu obstructie extrinseca de catre bronhie sau a. pulmonara)
Tip 2: cardiaca (25% din cazuri)- venele pulmonare dreneaza in sinusul coronar sau, mai rar in atriul drept (fara obstructie).
Tip 3: infracardiaca sau infradiafragmatica (20% din cazuri)- venele conflueaza intr-o vena verticala descendenta care trece in abdomen prin orificiul esofagian al diafragmului si se varsa in - vena porta (cu obstructie)
- ductul venos - vena cava inferioara
Tip 4: mixta (5% din cazuri)- venele pulmonare se varsa separat in locuri diferite
![Page 4: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/4.jpg)
DIAGNOSTIC
Ecografic , pe sectiunea cu 4 camere:
Figure 5.24 Anomalous pulmonary venous connection. (a) In a 4-chamber view obtained in a fetus with TAPVC, there is an atrioventricular disproportion, with the right sections larger than the left ones. AD, right atrium; AS, left atrium, VD, right ventricle; VS, left ventricle.(b) A venous confluence chamber (arrow) can be seen behind the left atrium (H, heart). (c) Spectral Doppler detects the typical pulmonaryvelocity waveform.
![Page 5: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/5.jpg)
SINDROMUL SCIMITAR
Congenital pulmonary venolobar syndrome
![Page 6: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/6.jpg)
Sindromul asociat cu PAPVR este cunoscut sub numele de SINDROM SCIMITAR dupa forma curba a venelor pulmonare drepte care se varsa in vena cava inferioara
scimitar= sabie curba
crearea unui sunt stanga-dreapta.
![Page 7: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/7.jpg)
![Page 8: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/8.jpg)
Prevalenta
Intre 1/100,000 si 1/33,333 nasteri. Sexul feminin pare sa fie mai afectat
decat cel masculin In majoritatea cazurilor, boala se
manifesta in primele luni de viata
![Page 9: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/9.jpg)
Chest x-ray of a five-year-old girl with Scimitar syndrome. The heart (blue outline) is shifted into
the right half of the chest, and the anomalous pulmonary venous return (red) has a shape
reminiscent of a Scimitar.
![Page 10: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/10.jpg)
Chest MR angiography in a patient with scimitar syndrome
![Page 11: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/11.jpg)
Se asociaza cu:
Dextrocardie Hipoplazie si malformatii arteriale
pulmonare drepte Malformatii cardiace (¼ din cazuri):
coarctatie de aorta; tetralogie Fallot, persistenta ductului arterial; DSV;
Chiste bronsice; plaman in potcoava, hernie diafragmatica
Sdr. ochilor de pisica (rar),
![Page 12: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/12.jpg)
ETIOLOGIE
Nu e complet cunoscuta Gena 4q de pe cromozomul 12 asociata
cu TAPVR
![Page 13: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/13.jpg)
DIAGNOSTIC
Prenatal: ecocardiografie fetala Clinic in perioada neonatala: cianoza,
dispnee, anurie, acidoza, insuficienta respiratorie, insuficienta cardiaca congestiva, infectii respiratorii recurente
Ecocardiografie transtoracica sau transesofagiana
Angiografie, CT, angioRMN
![Page 14: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/14.jpg)
DIAGNOSTIC DIFERENTIAL
Cauze de disproportie cardiaca: coarctatia de aorta
Sindrom pseudoscimitar – traseu si insertie anormala a venelor pulmonare in atriul stang
Sindromul Kartagener( AR, diskinezie ciliara- sinuzita cronica, bronsiectazie si situs inversus)
![Page 15: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/15.jpg)
TRATAMENT
Diagnostic prenatal: transfer in utero catre un centru tertiar de chirurgie cardiaca- trat. chirurgical in primele ore de viata in cazurile cu obstructie
TAPVC neobstructive- op. in primele 2 luni de viata
Operatia: restabilirea comunicarii venelor pulmonare cu atriul stang- sternotomie mediana in conditii de hipotermie si circulatie extracorporeala.
![Page 16: Sindrom Scimitar](https://reader031.vdocuments.mx/reader031/viewer/2022012310/55cfe37d5503467d968b496e/html5/thumbnails/16.jpg)
PROGNOSTIC, SUPRAVIETUIRE SI CALITATEA VIETII
Intrauterin, malformatia nu are consecinte hemodinamice decat daca se asociaza cu malf. cardiace
Postnatal, prognosticul depinde de asocierile malformative, prezenta obstructiei si precocitatea diagnosticului
In TAPVC izolate, in functie de tipul anatomic si operat precoce morbiditatea si mortalitatea este scazuta (1–10%) si speranta de viata este buna
Fara tratament-prognostic nefavorabil