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Atrijski septalni defekt (ASD) najčešća je srčana greška u odrasloj dobi. Često se kasno dijagnosticira. Potrebno je posumnjati na ovaj defekt kad se ehokardiografski registrira uvećana desna klijetka. Prikazana su dva slučaja pacijenata starijih od 50 godina s novodijagnosticiranim ASD-om. U obaju bolesnika greška se prezentirala s aritmijom ( brilacija atrija, odnosno totalni atrio-ventrikulski blok) i učinjena je uspješna kirurška, odnosno intervencijska korekcija defekta. Ako nema kontraindikacija, a najčešća je razvoj značajne plućne hipertenzije, odnosno plućne vaskularne rezistencije, preporučuje se aktivni stav, tj. zatvaranje defekta, jer znatno pridonosi kliničkom poboljšanju, ali i smanjenju mortaliteta., Atrial septal defects (ASD) are the most common heart defect in adult patients and are often diagnosed late. This defect should be suspected whenever echocardiographic imaging nds an enlarged right ventricle. We report two cases of patients with newly-diagnosed ASD that were older than 50 years of age. In both cases the patients presented with arrhythmia (atrial brillation, i.e. total atrioventricular block), and a successful correction of the defect was performed. If there are no contraindications, that is primarily the development of signi cant pulmonary hypertension i.e. pulmo- nary vascular resistance, the active approach – defect closure – is recommended, since it signi cantly contributes to clinical improvement and reduction in mortality.

Broj je bolesnika s prirođenim srčanim greškama u odrasloj dobi (PSGO) u porastu pa premda njihov točan broj i raspodjela u Hrvatskoj nisu poznati, potreba za njihovim praćenjem i liječenjem u specijaliziranom centru postaje nužnost. Prema procjeni radi se o više od 12.000 bolesnika kod kojih se u većini slučajeva očekuje neka od kasnijih komplikacija i potreba za jednom ili više procedura. U preporukama Europskog kardiološkog društva dane su smjernice za broj potrebnih centara, kao i za njihovu organizaciju. U Kliničkom bolničkom centru Zagreb započeta je organizacija centra. Opisani su prvi koraci koji uključuju organizaciju ambulante za PSGO, redoviti multidisciplinarni konzilij, kao i formiranje registra te prvi koraci u edukaciji kardiološke zajednice. U okviru Centra u kratkom je razdoblju pregledan velik broj bolesnika, što je omogućilo uvid u probleme te skupine bolesnika. Ovdje donosimo tri prikaza slučajeva. Prvi je pacijentica s operiranom tetralogijom Fallot kod koje nije pravilno dijagnosticirana i liječena aritmija, što može dovesti do znatnog kliničkog pogoršanja. Druga je pacijentica s neprepoznatom korigiranom transpozicijom, zbog manjka iskustva kardiologa. Iako pacijentica još nema značajnih tegoba, one se očekuju u budućnosti i stoga je postavljanje dijagnoze bilo ključno. U trećem slučaju prikazana je tipična PSGO pacijentica s multiplim operacijama u djetinjstvu kod koje se trajno traže nova rješenja; u ovom slučaju nekirurška implantacija zalistka na aortnoj poziciji. Ovaj prikaz ukazuje i na posebnost liječenja te skupne mladih ljudi kojima je osobito važna kvaliteta života., Due to the growing number of patients with adult congenital heart disease (ACHD), although their exact number and distribution in Croatia is not yet known, monitoring and treating these patients in specialized centers has become a necessity. Their number is estimated at 12 000, and in most cases these patients will require one or more surgical procedures due to complications later in life. The recommendations of the European Society of Cardiology include guidelines on the necessary number of specialized centers and their organization. Organization of such a center has begun in the Zagreb Clinical Hospital Center. This article describes the first steps being taken, which include setting up a clinic for ACHD, regular multidisciplinary councils, formation of a patient registry, and first steps towards educating the cardiologic community. A large number of patients were examined in the Center recently, providing insight into the specific problems in this patient population; we will present three cases here. The first is a woman with a surgically repaired tetralogy of Fallot but with an improperly diagnosed and treated case of arrhythmia, which can lead to serious clinical deterioration. The second patient is a woman with a case of corrected transposition that went undiagnosed due to a lack of experience on part of the cardiologist. Although the patient has not experienced significant difficulties, they are expected in the future, making a correct diagnosis extremely important. The third case is a typical ACHD patient, a woman that had undergone numerous surgeries in childhood in a constant search for a long-term solution, which in this case was a non-surgical valve replacement at aortic position. This overview also outlines specific challenges faced when treating young people who put especially high value on the quality of life.

cilj: Srcano ostecenje uzrokovano karcinoidnim sindromom je rijedak, ali zanimljiv i važan uzrok trikuspidalne i pulmonalne regurgitacije koje mogu dovesti do znacajnog morbiditeta i mortaliteta uzrokovanog zatajivanjem desnoga srca. Kada se stanje lijeci medikamentozno, i u adekvatnih kandidata kirurski, može se postici znacajna korist za ukupnu kvalitetu života i dugogodisnje preživljenje. Uloga ovoga prikaza slucaja je naglasiti važnost klinicke prosudbe u lijecenju pacijenata s karcinoidnim sindromom uz zahvacanje desnoga srca, kao i prikaz tipicnih i zanimljivih ehokardiografskih zapisa.

A 40-year-old male was admitted to our Clinic for evaluation of pulmonary arterial hypertension. Transthoracic (TTE) and transesophageal (TEE) two and three-dimensional echocardiography revealed obstructive membrane in the left atrium with small fenestration of only few millimeters, pulmonary artery pressure (PAP) of 60 mmHg and moderately dilated and hypertrophied right ventricle (RV). Late presentation led us to look for concomitant anomalies, so a partial anomalous left pulmonary venous connection was found. The diagnosis was confirmed by computed tomography (MSCT) — upper left pulmonary vein (LUPV) was drained into left brachiocephalic vein via vertical vein, and no lung anomalies were found. On coronary angiography, separate origins of the left anterior descending and circumflex artery were shown. The patient was referred to a cardiac surgeon, but he refused an operative correction at the time. Three years later, he was admitted because of dyspnea on exertion and clinical signs of right sided heart failure. On TTE, impairment of RV function was found, with severe RV dilatation and PAP of 90 mmHg. Cardiac magnetic resonance revealed no significant amount of RV fibrosis, and by right heart catheterization, pulmonary vascular resistance index (PVRI) of 1.45 Wood was calculated. Left-to-right shunt was estimated by radionuclide angiocardiography to 26-30%. After medical stabilization, surgical resection of intraatrial membrane and LUPV reconnection into the left atrium appendage was performed. One month after the operation, the patient was asymptomatic and positive remodeling of RV, normal flow in the left atrium and PAP of 23 mmHg were found. Cor triatriatum sinister is a very rare condition, especially in combination with anomalous pulmonary venous connection, so it is essential to search for concomitant anomalies. In the patient with signs of RV failure, multimodality approach was used to estimate the RV function. The calculation of PVRI was very important in making a decision whether this patient was operable. Some congenital causes of RV heart failure, such as cor triatriatum with anomalous pulmonary venous connection are important to be recognized in adults since they are surgically correctable due to low PVR. Apart from the diagnosis, multidisciplinary approach is also necessary in the assessment of RV function and PVR, key determinants in successful operation.