Thrombosis of a biological pulmonary valve in a young patient on anticoagulant therapy with rivaroxaban: a case report

Background: Patients with repaired tetralogy Fallot often develop severe pulmonary regurgitation (PR) and need surgical or catheter valve replacement/implantation. Early valve failure is not expected and thrombosis of a biological valve in a mid-term period after surgery on pulmonary position is rare. ----- Case summary: We report a 33-year-old female patient, who presented with heart failure, 18 months after surgical implantation of a biological valve on pulmonary position for severe PR, after previous complete repair. The patient was on anticoagulant therapy with novel oral anticoagulants (NOACs) for paroxysmal atrial fibrillation. After revealing a big pulmonary valve (PV) thrombus as a cause of severe valve stenosis and right heart failure, patient was re-operated without complication. After surgery a long-term warfarin therapy was introduced. The patient had an uneventful 9-month follow-up. ----- Discussion: Thrombotic events after rivaroxaban therapy are rare in non-valvular disease and there is paucity of data for NOAC therapy related to valve thrombosis. In our case, severe heart failure 1 year and a half after PV replacement, in a patient taking anticoagulant therapy, was unexpected. The diagnosis of valve thrombosis was revealed by echocardiography, and confirmed by computed tomography. We did not find any sign of thrombophilia, or any mechanical reason for valve thrombosis

Should we use CT or MRI for detection and characterization of benign adrenal lesions?

OBJECTIVES: Computed tomography (CT) and magnetic resonance imaging (MRI) are the main imaging modalities used for analysis of adrenal lesions. We compared the ability of CT and MRI to detect and characterize benign adrenal lesions. ----- PATIENTS AND METHODS: Unenhanced abdominal CT and MRI were performed in 16 patients (age range 39-77), and reviewed by a radiologist with 6years of experience in abdominal imaging. The presence, number, size and structure of each mass were analyzed and compared between the two modalities. ----- RESULTS: There were 18 adrenal masses in 11 patients, four patients had adrenal hyperplasia (AH), whereas one patient had left-sided AH and right-sided adenoma. Ten masses were≥2cm in diameter, and were perfectly depicted using CT and all MRI techniques. There were nine masses with diameter10Hounsfield units could not be characterized using unenhanced CT, but three of them were characterized using MRI. ----- CONCLUSION: CT has higher sensitivity for detection of small adrenal tumours and adrenal hyperplasia than MRI. MRI is an important tool in characterization of adrenal masses that could not be characterized using unenhanced CT

AORTO-LEFT VENTRICULAR TUNNEL – FETAL DIAGNOSIS AND EARLY NEONATAL TREATMENT AS A PRECONDITION FOR SUCCESFUL DISEASE OUTCOME

Prikazujemo pacijenta s aorto-lijevo ventrikulskim tunelom (ALVT, engl. aorto-left ventricular tunnel) kojemu je dijagnoza postavljena u gestacijskoj dobi (GD) od 27 tjedana. Indikaciju za pregled postavio je opstetričar zbog kardiomegalije. Ona se očitovala izrazitom dilatacijom lijeve klijetke (LVIDd 32 mm, >2SD), značajno smanjenom sposobnosti kontraktilnosti (EF 40 %) i obojenim doplerskim prikazom asimetrične insufi cijencije aortalne valvule putem kanala koji ide od sinotubularne konekcije do lijeve klijetke, između interventrikulskog septuma i subinfundibularnog trakta desne klijetke. Ventrikulska učinkovitost (engl. Myocardial Performance Index –MPI ili Tei indeks) bio je 0,62 (n.v. 0,25-0,50), kardiovaskularni profi l (engl. Cardiovascular Profi le Score – CVPS) prema Huhtai 4 (normalno 10), a-val u duktus venozus (DV) pozitivan (++) (kao siguran dokaz srčane insufi cijencije kod fetusa) i pozitivne pulsacije u umbilikalnim venama. Uz to je nađena ekstremna dilatacija tubularne aorte (18 mm – >2 SD, iznad 95. percentile). Prsten aortalne valvule u toj gestacijskoj dobi iznosi 5,8 mm (75. percentila), a širina ekstravalvularnog tunela 4-5 mm. Intrauterino se ne provodi medikamentno liječenje jer nije nađen hidrops. I postpartalno se nalazi kardiomegalija (rendgenska slika), a ehokardiografski potvrđuje dijagnoza ALVT-a. Kanal se nalazi iznad desnog koronarnog kuspisa, odnosno ispred rafe koja spaja lijevi i desni koronarni kuspis tako da se nalazi i dijagnoza bikuspidalne aortalne valvule (BAV) anteroposteriorne orijentacije. Zbog razvoja srčane insufi cijencije ordinira se inotropna i diuretska terapija. Dijagnoza je potvrđena i MSCT-om te kateterizacijom srca (angiokardiografi ja). Kardiokirurška operacija učinjena je u dobi od 16 dana postavljanjem autologne perikardijske zakrpe (engl. patch) na aortalni otvor kanala. Postpartalno se ordinira diuretik, digitalis i ACE inhibitor. Postupno dolazi do oporavka lijeve klijetke (3 mjeseca nakon operacije EF 50 %), ali perzistira patološka dilatacija tubularne aorte. Očekuje se potpuni oporavak lijeve klijetke uz potrebu daljnje opservacije bolesnika zbog bikuspidalne aortalne valvule s mogućim reperkusijama na samoj valvuli i uzlaznoj aorti. U dobi od 10 mjeseci nađena je uredna kontraktilnost lijeve klijetke (EF 65 %), blaga aortalna insufi cijencija (centralna), uz uredan protok kroz aortalnu valvulu, ali dalje postoji dilatacija uzlazne aorte (promjer 2,1 cm, >2 SD).We present a patient with aorto-left ventricular tunnel (ALVT) diagnosed in the gestational age (GA) of 27 weeks. Examination was indicated by an obstetrician due to cardiomegaly. ALVT manifested itself by an extreme dilatation of the left ventricle (LVIDd 32 mm, >2 SD), a signifi cantly decreased contractile ability (ejection fraction (EF) 40%) and color Doppler display of an asymmetric aortic valve insuffi ciency via a canal fl owing from the sinotubular junction to the left ventricle, between the interventricular septum and subinfundibular portion of the right ventricle. Myocardial performance index was 0.62 (normal 0.25-0-50), cardiovascular profi le score 4 (normal 10), a-wave in ductus venosus (DV) positive (++) (as positive evidence for fetal cardiac dysfunction) and positive umbilical venous pulsations. In addition, an extremely dilated tubular aorta was also found (18 mm – >2 SD, above 95th percentile). In that gestational age, aortic valve ring was measured 5.8 mm (75th percentile), and the width of extravalvular tunnel 4-5 mm. Since no hydrops was found, in utero treatment with medicines was not used. Cardiomegaly was found postpartum (x-ray), and the ALVT diagnosis was confi rmed by echocardiography. The canal was located above the right coronary cusp, i.e. in front of the raphe connecting the left and right coronary cusp, meaning that the diagnosis of a bicuspid aortic valve (BAV) of anterior-posterior orientation was also made. Inotropic and diuretic therapy was ordered due to cardiac insuffi ciency development. Diagnosis was also confi rmed with multi-slice computed tomography and heart catheterization (angiocardiography). Cardiac surgery was performed at the age of 16 days by placing an autologous pericardial patch on the aortic canal opening. Diuretic, digitalis and angiotensin-converting enzyme inhibitor were ordered postnatally. Left ventricle was recovering incrementally (3 months after surgery, EF 50%), but pathological dilatation of tubular aorta persisted. Complete recovery of the left ventricle is expected, with the need for further observation of the patient because of the BAV and possible effects on the valve itself and on the ascending aorta. At the age of 10 months, contractility of the left ventricle was normal (EF 65%), with mild aortic insuffi ciency (central), normal fl ow through the aortic valve, but dilatation of the ascending aorta persisted (ascending aorta width 2.1 cm, >2 SD)

Multiple Structural Failures of Aortic Homografts Leading to Emergency Surgery

Aortic homografts are composed of aortic roots and variable lengths of the arch and ascending aorta, harvested from donor hearts. They are primarily used in extensive aortic root endocarditis due to its resistance to reinfection. They are, however, prone to structural failure. Redo aortic root surgery is challenging, especially in the setting of aneurysms or pseudoaneurysms (PSAN) lying immediately posterior to the sternum or adhering to it. We present a 43-year-old female with two aortic homograft pseudoaneurysms following her fourth aortic valve and ascending aortic procedure. The first PSAN was described as immediately cranial to proximal homograft anastomosis, measuring 21x8 millimeters. It harbored potential for intraoperative rupture because of its proximity to the posterior sternal table. The second one was 36x20 millimeters, adjacent to the distal homograft anastomosis. The surgical plan entailed institution of peripheral hypothermic cardiopulmonary bypass due to an almost inevitable risk of bleeding from the retrosternal pseudoaneurysm. While the actual sternotomy was uneventful, the PSAN ruptured after sternal retraction. The ensuing massive bleeding was expected and was success- fully managed during hypothermic circulatory arrest. The patient had an unremarkable postoperative course and was discharged home

DJEVOJKA S TAKAYASU ARTERITISOM PROGRESIVNOG TIJEKA S MULTIPLIM KIRURŠKIM INTERVENCIJAMA

Takayasu arteritis is a rare large-vessel vasculitis but it can be associated with high mortality rates in childhood. Granulomatous vasculitis usually affects the aorta and/or main branches but also coronary and pulmonary arteries. The course of the disease is unpredictable and management is based on controlling infl ammation and preventing end-organ damage. In this case we describe a patient with progression of vasculitis but so far successful prevention of possible ischemic consequences using immunosupressive and biologic therapy and multiple surgical interventions over the course of the disease. A 14-year-old girl presented with precordial pain and numbness of the left arm. Physical examination revealed the absence of the radial pulse in the left arm. Computed tomography angiography showed subtotal occlusion of the left main coronary artery, subtotal occlusionof the left common carotid artery, subtotal occlusion of the left subclavian artery and stenosis of thoracic aorta below isthmus of aortae. Despite aggresive conservative therapy and cardiosurgical treatment the course of the disease was complicated with restenoses which were resolved with subsequent revascularization procedures. Here we present an adolescent girl with progressive vasculitis and with multiple surgical interventions. Carefully monitoring of the patient and good collaboration between pediatric cardiologist and rheumatologist with radiologists and cardiac surgeons improved life-quality of the patient which now studies at the University and has good physical and mental status.Takayasu arteritis je vaskulitis velikih krvnih žila koji zahvaća aortu i njezine ogranke ali i pulmonalne i koronarne krvne žile. Tijek bolesti je nepredvidiv i terapija obuhvaća smirivanje upalnog procesa i prevenciju oštećenja organa. Prikazujemo bolesnicu s progresivnim tijekom bolesti koji je zasada uspješno obuzdavan uz pomoć imunosupresivne i biološke terapije kao i multiplim kirurškim postupcima. 14-godišnja djevojčica se očitovala prekordijalnim bolovima i utrnućem lijeve ruke s gubitkom pulsa radijalne arterije. CT angiografi jom je nađena totalna okluzija debla lijeve koronarne arterije, subtotalna okluzija lijeve zajedničke karotidne arterije i lijeve potključne arterije te stenoza torakalne descendentne aorte ispod razine istmusa. Unatoč agresivnoj konzervativnoj terapiji i kardiokirurškom tretmanu daljnji tijek bolesti je kompliciran restenozama koje su razriješene postupcima revaskularizacije. Pažljivim praćenjem bolesnice kao i dobrom suradnjom pedijatrijskih kardiologa i reumatologa s kardiokirurzima te radiolozima omogućena je dobra kvaliteta života u djevojke koja se uspješno školuje te je u dobrom fi zičkom i mentalnom stanju

DJEVOJKA S TAKAYASU ARTERITISOM PROGRESIVNOG TIJEKA S MULTIPLIM KIRURŠKIM INTERVENCIJAMA

Takayasu arteritis is a rare large-vessel vasculitis but it can be associated with high mortality rates in childhood. Granulomatous vasculitis usually affects the aorta and/or main branches but also coronary and pulmonary arteries. The course of the disease is unpredictable and management is based on controlling infl ammation and preventing end-organ damage. In this case we describe a patient with progression of vasculitis but so far successful prevention of possible ischemic consequences using immunosupressive and biologic therapy and multiple surgical interventions over the course of the disease. A 14-year-old girl presented with precordial pain and numbness of the left arm. Physical examination revealed the absence of the radial pulse in the left arm. Computed tomography angiography showed subtotal occlusion of the left main coronary artery, subtotal occlusionof the left common carotid artery, subtotal occlusion of the left subclavian artery and stenosis of thoracic aorta below isthmus of aortae. Despite aggresive conservative therapy and cardiosurgical treatment the course of the disease was complicated with restenoses which were resolved with subsequent revascularization procedures. Here we present an adolescent girl with progressive vasculitis and with multiple surgical interventions. Carefully monitoring of the patient and good collaboration between pediatric cardiologist and rheumatologist with radiologists and cardiac surgeons improved life-quality of the patient which now studies at the University and has good physical and mental status.Takayasu arteritis je vaskulitis velikih krvnih žila koji zahvaća aortu i njezine ogranke ali i pulmonalne i koronarne krvne žile. Tijek bolesti je nepredvidiv i terapija obuhvaća smirivanje upalnog procesa i prevenciju oštećenja organa. Prikazujemo bolesnicu s progresivnim tijekom bolesti koji je zasada uspješno obuzdavan uz pomoć imunosupresivne i biološke terapije kao i multiplim kirurškim postupcima. 14-godišnja djevojčica se očitovala prekordijalnim bolovima i utrnućem lijeve ruke s gubitkom pulsa radijalne arterije. CT angiografi jom je nađena totalna okluzija debla lijeve koronarne arterije, subtotalna okluzija lijeve zajedničke karotidne arterije i lijeve potključne arterije te stenoza torakalne descendentne aorte ispod razine istmusa. Unatoč agresivnoj konzervativnoj terapiji i kardiokirurškom tretmanu daljnji tijek bolesti je kompliciran restenozama koje su razriješene postupcima revaskularizacije. Pažljivim praćenjem bolesnice kao i dobrom suradnjom pedijatrijskih kardiologa i reumatologa s kardiokirurzima te radiolozima omogućena je dobra kvaliteta života u djevojke koja se uspješno školuje te je u dobrom fi zičkom i mentalnom stanju