Bir yaş altı, vetriküler septal defekt nedeni ile opere edilen pulmoner hipertansif hastalarda iloprost kullanımının hemodinamik parametrelere etkisi

Bu çalısmada, ventriküler septal defekt (VSD) nedeni ile opere edilen, pulmoner hipertansif, bir yasının altındaki hastalarda postoperatif iloprost kullanımının hemodinamik paremetrelere etkisi incelenmistir. Çalısma hastaları iloprost alan ve almayan olmak üzere iki ana gruba, iloprost alan hastalar ise intraoperatif iloprost baslanılanlar ve pulmoner hipertansif (PH) kriz sonrası baslanılanlar olmak üzere iki alt gruba ayrılmaktadır. Hastaların yas, kilo, vücut kitle indeksi gibi demografik bilgilerinde anlamlı bir fark saptanmamıstır, bu özelliği literatürdeki diğer çalısmalardan ayrılmasını sağlamaktadır. Hastaların preoperatif kardiyak kateterizasyonlarından elde edilen ortalama ve sistolik pulmoner arter basınçları, pulmoner vasküler direnç indeksleri, akımlar oranları, sistemik oksijen saturasyonları arasında istatistiksel olarak anlamlı bir fark saptanmamıstır. Hastaların cerrahi sürelerinde anlamlı bir farklılık saptanmamıstır ancak kardiyopulmoner bypass çıkısında ölçülen pulmoner arter basınçları, iloprost tedavisinden bağımsız olarak, postoperatif PH kriz geçiren ve eksitus olan hastalarda istatistiksel olarak anlamlı derecede yüksek bulunmustur. Postoperatif pulmoner arter basınç ortalamasının PH kriz geçiren hastalarda ve PH kriz sonrası iloprost baslanılan hastalarda yüksek olarak bulunmasının yanında iloprost almayan ve intraoperatif iloprost baslanılan hastaların değerleri arasında fark saptanmamıstır. PH kriz sonrası iloprost baslanılan hastalarda iloprost infüzyonu sonrasında ortaya çıkan PH kriz sayısı diğer gruplara kıyasla anlamlı derecede yüksektir. Ayrıca intraoperatif olarak iloprost baslanılan hastalarda baslanılmayanlara göre istatistiksel olarak anlamlı bulunmayan ancak yüzde değerlerinde belirgin derecede daha az pulmoner hipertansif kriz saptanmıstır. Hastaların postoperatif pozitif inotrop desteklerinde farklılık saptanmazken, ekstübasyon, yoğun bakım ve hastanede kalıs süreleri iloprost alan hastalarda daha uzun olarak saptanmıstır. ĐV iloprost uygulamasında görülen, sistemik kan basıncında düsüs, hastalarda gözlenmis olsa da, ek önlemler gerektirmemistir. Hastaların postoperatif ilk bir ay içerisinde yapılan ekokardiyografilerinde, gruplar arasında, persistan pulmoner hipertansiyon açısından anlamlı bir fark saptanmamıstır. Retrospektif olarak tasarlanan arastırmamızda elde edilen sonuçlar, iloprostun postoperatif erken dönem pulmoner hipertansiyon ve PH krizin önlenmesi için, riskli hasta gruplarında intraoperatif olarak baslanmasının daha avantajlı olabileceği yönünde destekleyici bulgular vermektedir. Ancak, sonuçlar üzerinde daha kesin yorum yapılabilmesi için prospektif çalısmalar ile doğrulanması gereklidir. In this study, we examined the effect of iloprost on postoperative hemodynamic paremeters in children under the age of one year, with pulmonary hypertension and VSD. There were two main groups, one is the patients who had the iloprost therapy, and the patients who didn’ t have it, and the iloprost therapy group was divided into two subgroups as the patients to whom iloprost was started intraoperatively and iloprost was started after a pulmonary hypertensive (PH) crisis. There was no difference in the demografic datas of the patients as age, body mass, body mass index, this pecularity differs our study from the litretaure. There was no statistical difference between groups in the datas taken from the preoperative cardiac cateterization as mean and systolic pulmonary pressure, pulmonary vascular resistance index, flow rate and systemic oxygen saturation. There was no difference in operating times, but the pulmonary artery pressures at the end of the cardiopulmonary bypass, independently from iloprost, was statistically higher in the patients with postoperative PH crisis and in the exitus group. Postoperative pulmonary artery pressures were higher in the patients with PH crisis, there was no difference between the patients without iloprost therapy and the patients that iloprost started intraoperatively, but postoperative pulmonary artery pressures were significantly higher in the group of iloprost was started after PH crisis group. In the group of iloprost started after a pulmonary hypertansive crisis, the number of PH crisis occured after iloprost therapy initiation was higher than other groups. Đn the patient group of iloprost was started intraoperatively, percentage of patients with PH crisis is significantly lower than other patients whom iloprost was’ st started intraoperatively, even statistical analyses does not indicate significant differance. The most important adverse effect of iloprost in the litreture was systemic hypotension, which was not observed in our study. There was no difference in postoperative pozitive inotropic supports, but in the iloprost group extubation, intensive care unit and hospital stay durations were longer. There was no difference betweeen groups about postoperative persistant pulmonary arterial hypertension in the echocardiographic examinations performed in the first month after operation. Our study, planned as a retrospective study, suggests supportive findings about that iloprost therapy can have better results, in the postoperative period to avoid PH crisis and postoperative persistant pulmonary hypertension, if started intraoperatively to the patients with pulmonary hypertension and VSD. But prospective studies must be performed to make definite comments on our findings

Premature Coronary Artery Disease due to Homozygous Familial Hypercholesterolemia in a 12-Year-Old Girl

Background: Homozygous familial hypercholesterolemia is a rare inherited metabolic disease caused by low-density lipoprotein receptor abnormality. Patients with homozygous familial hypercholesterolemia have an increased risk of cardiovascular complication that usually occurs in the first decade of life. Here, we report a 12-year-old girl with an unpredicted presentation for coronary artery disease and found to have homozygous familial hypercholesterolemia. Case Report: A 12-year-old girl was admitted to our unit with syncope. Chest X-ray showed bilateral diffuse pneumonic consolidation and mild cardiomegaly. We detected stable ST depression by electrocardiography. Echocardiography showed normal systolic functions. Troponin-1 levels were high (66 mcg/dL, upper limit: 0.04 mcg/dL). Influenza A virus DNA was detected by the respiratory viral panel. After her successful treatment for acute pneumonia and myocarditis due to Influenza A virus, her syncope attacks persisted. Marked ST elevation was observed during exercise electrocardiography. Coronary angiography showed severe occlusions in the coronary arteries. High serum levels of total cholesterol (756 mg/dL) and low-density lipoprotein-C (556 mg/dL) were noticed. She had no tendon xanthomas. Medical histories revealed that her family members were diagnosed with heterozygous familial hypercholesterolemia. A coronary bypass surgery was performed. Statin and ezetimibe treatments were started. We also planned lipid apheresis. Conclusion: Children with homozygous familial hypercholesterolemia may present with symptoms of premature coronary heart disease requiring a routine lipid test and careful anamnesis

Cardiovascular Surgery and Interventions Case Report Open Access Coronary artery bypass graft surgery in a pediatric patient with a giant coronary aneurysm

Kawasaki disease or mucocutaneous lymph node syndrome is an acute, febrile vasculitis of unknown etiology affecting mainly infants and children under five years of age. Coronary artery involvement, which occurs in approximately 20% of such patients, may be the most important feature of this syndrome. In this paper, we report a successful coronary artery bypass graft in a nine-year-old boy with a giant coronary artery aneurysm and diffuse coronary artery disease. Keywords: Aneurysm; coronary artery bypass grafting; Kawasaki disease. Kawasaki disease (KD), previously known as mucocutaneous lymph node syndrome, is an acute, febrile, self-limiting generalized vasculitis of unknown etiology which occurs predominantly in infants and young children. It mainly affects small-and medium-sized arteries, particularly the coronary arteries, and was described by Kawasaki in 1967. [1] Gulhan et al. [2] reported a 42.4% incidence of coronary involvement in Turkish patients with KD. Kawasaki disease causes aneurysm formation at the proximal sites of the coronaries, while the distal sites are usually protected. Dilatation equal to or larger than 8 mm is categorized as a giant aneurysm, which may cause coronary artery stenosis secondary to compression or thrombosis. [3] Early diagnosis and rapid therapeutic interventions such as aspirin and intravenous gamma globulin may decrease the risk of coronary artery pathology development by approximately 20%. CASE REPORT A nine-year-old boy with KD was admitted to our institution with complaints of chest pain and fatigue. The patient's history revealed KD. He experienced the first attack at the age of two with prolonged fever. Neither intravenous gamma globulin nor steroid therapy was given. Laboratory examination showed leukocytosis, high C-reactive protein, and elevated liver enzymes. Echocardiography showed pancarditis and a giant calcified and thrombotic coronary artery aneurysm. The diameter of the left main coronary artery aneurysm was measured as 40 mm. The patient was enrolled in a treadmill exercise test according to the modified Bruce protocol. Ischemic signs were found in the anterior leads. Coronary angiography revealed a giant left main coronary artery aneurysm, a totally occluded left anterior descending artery, and a diffusely diseased circumflex arter