Cor Triatriatum İle Birlikte Bulunan Bir Tricuspid Atresia’sı Vak’ası

Year 1967, Volume: 20 Issue: 2, 292 - 308, 30.06.1967

Binnaz Ege

Abstract

Cor triatriatum ile birlikte bulunan bir tricuspid atrezisi vakası takdim edilmiştir. Gerek kor triatriatum gerekse trikuspid atrezisi başlı başına nadir rashanan konjenital kalp hastalıklarından olup bu iki anomalinin aynı vak’ada bulunuşuna şimdiye kadar yaptığımız araştırmalara göre rashamadığından vak’ayı takdim etmeyi uygun bulduk.

Hasta 2.5 aylık erkek çocuğu olup ağır dispne ve syanoz şikâyetleri ile kliniğe yatırılmıştır. Muayenede, sternumun solunda 3 - 4 üncü interkostal aralıkta 4 üncü dereceden haşin pansistolik bir sufl, jugular venlerde ve karaciğerde presistolik venöz pulsasyon EKG de sol axis deviasyonu, sol ventrikül hipertrofisi ve P pulmonale tesbit edildiğinden klinik olarak tricuspid atrezisi teşhisi konulmuştur. Çocuk hastahanede yattığı süre esnasında günde 2 - 3 kere anoksik nöbet ve kalp iflası belirtileri göstermiş ve bu nöbetlerden biri esnasında ölmüştür. Otopside Edwards ve Burchell’in klassifikasyonuna göre Tip lb triktispid atrezisi ve bununla birlikte sol atriumun superior dorsal kısmında lokalize olmuş diğer bir chamber yani kor triatriatum tesbit edilmiştir 4 pulmoner vende bu aksesuar chamber’a agliyordu ve bu aksesuar chamber ile esas sol atrium arasındaki intraatrial septumda takriben bir cm. gapında bir açıklık mevcuttu. Foramen Ovale açık olup ağ atrium ile esas sol atrium arasında yer almıştı. Mitral valv tamalle normaldi. Aksesuar chamber’ın mikroskopik muayenesinde atrium adele lifleri tesbit edildi.

Keywords

Tricuspid atrezisi, Cor triatriatum, Konjenital kalp hastalığı

Ethical Statement

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Supporting Institution

Ankara Üniversitesi Tıp Fakültesi Pediatri Kliniği

Project Number

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Thanks

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A Case Of Tricuspid Atresia Associated With Cor Triatriatum

Abstract

A case of tricuspid atresia associated with cor triatriatum have been described. This case illustrated two unusual pathologic features. Namely, tricuspid atresia and cor triatriatum. Both of them are rare congenital cardiac malformations. Because of the combination of these two malformations in one case have not been found in the world literature, the case has been presented.

The baby was 2.5 months old girl who was admitted to the hospital because of severe cyanosis and dyspnea. Cyanosis was present since birth. Fourth degree harsh, pansystolic murmur was audible at the 3rd and 4th left intercostal space. Presystolic venous pulsation was noted at the jugular veins at the liver edge. EKG showed left axis deviation, left ventricular hypertrophy and P pulmonale. Tricuspid atresia was diagnosed clinically. The child noticed 2 - 3 anoxic spells in a day during his stay in the hospital congestive heart failure developed. Because of very poor general conditions and repeated anoxic spells, angiocardiography has not been done.

The child died during one of the anoxic spells and autopsy performed.

According to Edwards and Burchell classification, Type Ib tricuspid atresia has been found. This anomaly also has been associated with accessory chamber located at the superior dorsal part of the left atrium, namely cor triatriatum. Four pulmoner veins has been opened to this accessory chamber. There was one hole approximately one Cm. in diameter at the intraatrial septum and mitral valve was completely normal. Foramen ovale was patent and located between the right atrium and the real left atrium. On microscopical examination accessory chamber showed atrial muscles.

Keywords

Tricuspid atresia, Cor triatriatum, Congenital heart disease

Ethical Statement

-

Supporting Institution

Ankara University Faculty of Medicine Pediatrics Clinic

Project Number

-

Thanks

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References

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