İzole atrial septal defekt, ventriküler septal defekt, patent duktus arteriozusun klinik seyri

Abstract

ÖZET İzole ASD, VSD ve PDA'nın klinik seyrini ve buna etki eden faktörleri belirlemek için 1991-1998 yılları arasında DEÜTF Pediatrik Kardiyoloji Bilim Dalına başvuran, tanı konup izleme alındığında 0-2ay arasında olan 77 izole ASD, 350 izole VSD, 40 izole PDA'lı hasta (l.grup), ve 2ay-18 yaş arasında izleme alınan 293 ASD, 792 VSD, 68 PDA'lı hasta (ll.grup) çalışmaya alınmıştır. Tanı anamnez, fizik muayene, telekardiyografi (tele), elektrokardiyografi (EKG), ekokardiyografi (Eko) ile konmuştur. Cinsiyet, geliş şikayetleri, ağırlık, boy, oskültasyon, Tele, EKG, Eko bulguları, klinik seyir, gelişen komplikasyonlar, kendiliğinden kapanma oranları değerlendirilmiştir. Ayrıca her iki gruptan toplam 189 (69 ASD, 52 VSD, 68 PDA'lı) hastada kardiyak kateterizasyonla belirlenen hemodinamik bulgular değerlendirilmiştir. Grup l'de yaşları 2-58 gün (ortalama 35 ± 21 gün) olan izole ASD'li 77 hasta 308 hasta yılı (ortalama 4 yıl) izlenmiş ve 13 hastada (%17'sinde) kendiliğinden kapanma saptanmıştır. 77 hastadan iksinde (%2.5'inde) koinsidantal olduğunu düşündüğümüz dilate kardiyomiyopati gelişmiş ve tıbbi tedavi ile gerilemiştir. Defekti kapanmayan 2 ile 4 yaş arasındaki 64 hastadan 33'üne (%61'ine) kateterizasyon uygulanmış 2 yaş, 3 yaş ile 3.5 yaşlarındaki 3 hastada (%9'unda) hafif pulmoner hipertansiyon (PH) saptanmıştır. Klinik izlemde bu grupta başka komplikasyon gelişmediği saptanmıştır. Grup ll'deki izole ASD'li 293 hastanın klinik izleminde bronkopnömoni nedeniyle 1 hasta (%03) kaybedilmiştir. Bu grupta 36 hastaya uygulanan kalp kateterizasyonunda yaşları 16ay - 5yaş arasında 26 hastada (%72) hafif PH, 8ay-13 yaşlarında 2 hastada (%5.5) ağır PH belirlendi. Bunlardan 8 aylık olan hastada ağır pulmoner vasküler direnç (PVD) artışı saptanmaştır. Grup l'de yaşları 2-58 gün (ortalama 25 ± 16 gün) olan 350 izole VSD'li hasta 1406 hasta yılı (ortalama 4,02 yıl) izlenmiş, 161 hastada (%46'sında) VSD kendiliğinden kapanmıştır. Küçük muskuler VSD'lerin hepsi kapanmış, geniş VSD'lerden ise hiçbiri kapanmamıştır. 350 hastadan 2'si (%06) bronkopnömoni nedeniyle kaybedilmiştir. Ayrıca 28 hastada (%8) orta veya ağır derecede TY, 25 hastada (%7) AVP, 15 hastada (%4) AY, 16 hastada (%4.6) hafif PS, 2 hastada (%06) cerrahi müdahale gerektirecek kadar ağır PS, 9 hastada (%3) subaortik8 stenoz, 22 hastada (%6) MY saptanmıştır. II.Gruptaki 792 izole VSD'li hastanın 3'ünde (%04) ağır bronkopnömoni gelişmiş ve bunlardan 2'si (%03) kaybedilmiştir. 6 yaşındaki bir hastada (%01) endokardit gelişimiş ve tıbbi tedavi ile iyileşmiştir. Grup I ve ll'deki izole VSD'li hastalardan 3ay-18yaş arasındaki 52 hastaya kalp kateterizasyonu uygulanmış, bunlardan 11 'inde (%21) hafif PH, 27'sinde (%52) ağır PH, 12'sinde (%23) hafif PVD artışı, 15'inde (%29) ağır PVD artışı saptanmıştır. Grup l'de yaşları 2gün-58 gün (ortalama 40 ± 20 gün) olan izole PDA'lı 40 hasta 164 hasta yılı (ortalama 4,1 yıl) izlenmiş ilk hafta içinde 8 hastada (%20) kendiliğinden kapanma saptanmıştır. 2'mm'den küçük çapta olan PDA'ların kapanma olasılığı diğerlerinden daha fazlaydı (p=0.01). Kendiliğinden kapanmayan hastalardan birinde (%2.5) 4 aylıkken ağır bronkopnömoni gelişmiş ve tıbbi tedaviyle düzelmiştir. Grup I ve ll'de kalp kateterizasyonu uygulanan toplam 68 izole PDA'lı hastadan 6ay-15yaş arasındaki 26 hastada (%38) hafif PH, 16ay-18yaşlar arasında 15 hastada (%22) ağır PH, 9ay-3yaş arasındaki 11 hastada (%16) hafif PVD artışı, 1yaş-18 yaş arasındaki 6 hastada (%9) ağır PVD artışı saptandı. Anahtar Kelimeler : Atrial septal defekt (ASD), ventriküler septal defekt (VSD), patent duktus arteriozus (PDA), klinik seyir.

CLINICAL COURSE OF ISOLATED ATRIAL SEPTAL DEFECT, VENTRICULAR SEPTAL DEFECT AND PATENT DUCTUS ARTERIOSUS During 1991-1998 77 isoleted ASD, 350 isoleted VSD and 40 isoleted PDA patients (group I) and 293 ASD, 792 VSD, 68 PDA patients (group II) were diagnosed and studied in order to determine the clinical course of these entities and the factors affecting it. Group I patients consisted of 467 cases. 297 of these cases were studied retroceptively and 170 were studied prospectively. All the patients were mature babies and age range was 0 to 2 months at the time of the first diagnosis. Group II patients age range was 2 months to 18 years at the time of the first diagnosis and all the patients were studied retroceptively. The diagnosis was establised using history, physical examination, teleradiography (tele), electrocardiography (EKG) and echocardiography (echo). Sex, symtoms at admission, body weight, oscultation findings, tele, EKG, eko results, complications developing during the follow up period, and spontaneous closure rates were determined and evaluated. The heamodinamic findings were measured and evaluated in 69 ASD, 52 VSD and 68 PDA patints whose diagnosis was establised at the age of 0-18 years (In group I and group II). 77 patients with isolated ASD and age range of 2-58 days (mean 35± 21 days were followed up for a period of 308 (mean 4) patient years and spontaneous closure was observed in 13 patients (17%). Dilated cardiomyopathy developed in 2 of the above 77 cases (2.5%) and it improved following medical tratment. We think the development of this entity was coincidental. Catheterization was carried out in 33 (61%) of 64 patients, age range 2-4 years, whose defects hadn't closed. In three cases (9%) at the age of 2, 3 and 3.5 years, a slight that no other complication had developed in this group. The clinical follow-up of 293 patients with isoleted ASD in group II showed that a patient had died of bronchopneumonia. Caheterization was carried out in 36 patients in this group. In 26 of these, age range 16 months-15years, a slight PH in 2 patients, 1 eight-month and other thirteen year old serious PH, and in 1 eight-month old patient (2.7%) a serious increase in pulmonary vascular resistance (PVR) were observed. In group I which consisted 350 VSD cases, age range of 2-58 days (mean 25±16 days) were observed for a period of 1406 (mean: 4,02) patient years. It was seen that in 161 patients (45%) in this group of VSD had closed n10 spontaneosly. All of the small VSDs closed; however, none of the large ones did. 2 of the 350 patients (0.6%) died of bronchopneumonia. Additonally, medium or serious TR in 28 patients (8%), AVP in 25 patients (7%), AR in 15 patients (4%), a slight PS in 16 patients (4.6%), serious PS necessitiating surgical intervention in 2 patients (0.5%), subaortic stenosis in 9 patients (3%) with an age range of 2.5 to 6 years, and MY in 22 patients (6%) were diagnosed. In group II which consisted of 792 VSD cases, three patients (0.04%) developed serious bronchopneumonia. They were twenty months, two and a half years and four years old each. Two of these three patients died of bronchopneumonia. A six-year-old patient developed endocardit (0.1%) and it improved with treatment. Of the isoleted VSD patients who had come to hospital with an age range of 0 to 18 years, 52 patients whose age range was 3 months to 18 years underwent catheterization 11 patiens (21%) with an age range of four months to 17 years developed slight PH, 27 patiens (52%), age range three months to thirteen years developed serious PH, 12 patients (23%) age range three months to thirteen years, defeloped a slight PVR increase, 15 patiens (29%) age range 4 to 6 years developed serious PVD increased. 40 patients with isolated PDA, age range 2days to 58 days (mean 40 ±20 days), were followed up for a period of 164 (mean 4.1) patient years and spontaneous closure was obreved in 8 patients (20%) witin the first week. Those with a diameter of less than 2 mm in echo had a higer possibility of spontaneous closure (p=0.01). In one of the patients bronchopneumonia developed at the age of four months and was cured with medical treatment. The haemodynamic findings of the 68 patients with isolated PDA and age range of 0 to 18 years at the time of their first examination showed 26 patients (38%), age range 6 months to 15 years, had a slight PH, 15 patients (22%) with an age range of 16 months to 18 years had serious PH, 11 patients (16%) with an age range of 9 months to three had a slight increase in PVD and 6 patients (9%) with an age range of 1 year to 18 years had serious PVD increase. Key words: Atrial septal defect (ASD), ventricular septal defect (VSD), patent ductus arteriosus (PDA), clinical curse. <i«