Advanced Therapy in the Treatment of Pulmonary Arterial Hypertension in Adult Congenital Heart Disease

Plućna arterijska hipertenzija (PAH) teška je kronična, progresivna bolest. Pojavljuje se u dijela bolesnika s prirođenim srčanim bolestima (PSB), a izražen je primjer Eisenmengerov sindrom, PSB s najvećim mortalitetom i morbiditetom. Napredna terapija PAH-a u prirođenim srčanim bolestima odraslih (PSBO-om) znatno je poboljšala ishod i klinički tijek u ovakvih bolesnika, a kao terapija prve linije danas su u preporukama antagonisti endotelin receptora, prije svega bosentan. U svim studijama i registrima bosentan je poboljšao toleranciju napora, reducirao plućnu vaskularnu rezistenciju i smanjio smrtnost takvih bolesnika. Ostali lijekovi napredne terapije (inhibitori fosfodiesteraze i prostaglandini) dodaju se u slučaju progresije bolesti. Napredna terapija PAH-a može se primjenjivati dugoročno s dobrim rezultatom i malo nuspojava. Osim Eisenmengerova sindroma i drugi bolesnici iz grupe PAH u PSBO mogu biti liječeni naprednom terapijom. U liječenju operiranih bolesnika s PSBO-om koji imaju ili se u njih naknadno razvije PAH indiciran je i drugi dvostruki inhibitor endotelina, macitentan. Terapija kombinacijom lijekova nije dovela do daljnjega bitnog poboljšanja u usporedbi s liječenjem monoterapijom inhibitorima endotelinskih receptora. Moguće je da će i pacijenti s Fontanovom cirkulacijom biti ciljana skupina za liječenje tim lijekovima.Pulmonary arterial hypertension (PAH) is a severe chronic, progressive disease. It affects the group of patients with congenital heart diseases (CHD); a high-profile example is Eisenmenger’s syndrome, the CHD with the greatest mortality and morbidity. Advanced PAH treatment for adult congenital heart diseases (ACHD) has significantly improved the outcome and clinical course for these patients, and endothelin receptor antagonist, primarily bosentan, are recommended as first-line treatment today. All case studies and registries show that bosentan improved the exercise tolerance, reduced pulmonary vascular resistance, and decreased the mortality of these patients. Other advanced therapy drugs (phosphodiesterase inhibitors and prostaglandins) are added in case of disease progression. Advanced PAH therapy can be administered long-term with good results and few side effects. Apart from Eisenmenger’s syndrome, other patients from the PAH group in ACHD can be treated through advanced therapy. In the treatment of surgical patients with ACHD who have developed or are developing a subsequent PAH, a different double endothelin inhibitor is indicated, macitentan. Therapy through a combination of drugs has not led to any further significant improvements in relation to treatment by a monotherapy of endothelin receptor inhibitors. It is possible that patients with Fontan circulation will be the target group for the treatment with these drugs

Thoracic EndoVascular Aortic Repair in the University Hospital Centre Zagreb: Ten Years since the first Intervention, problems and Results

Torakalni endovaskularni postupak postavljanja aortnog stent-grafta (TEVAR) razvijen je isprva za postupke okluzije degerativnih aneurizmi descendentne aorte, a uskoro se proširio na cijeli spektar patologije ovog aortnog segmenta, uključujući disekcije i traumatske rupture. Kirurško rješavanje ove patologije ima iznimno visok mortalitet i morbiditet te je TEVAR znatno poboljšao liječenje ovakvih bolesnika. U Kliničkom bolničkom centru (KBC) Zagreb upravo se zbog toga započelo s postupkom u okviru Klinike za bolesti srca i krvnih žila, i unatoč poteškoćama, metodom su liječeni najrizičniji bolesnici s aneurizmom, disekcijom tipa B i traumatskom rupturom descendentne aorte. Dugoročni su rezultati liječenja 24 bolesnika prihvatljivi, uz mortalitet od 8,3 % te uobičajene komplikacije (propuštanje tipa I. i II. u 12,5 %; infekcija u 4,1 %; proksimalno proširenje disekcije 4,1 %) koje su riješene dodatnom intervencijom ili operacijom. U budućnosti očekujemo mnogo brži razvoj metode u KBC-u Zagreb, ali i u Hrvatskoj općenito, nakon što je Hrvatski zavod za zdravstveno osiguranje riješio financiranje uređaja.Thoracic EndoVascular Aortic Repair (TEVAR) was initially developed for occlusion pro- cedures in treatment of diseases of the descending aorta, but its use was soon expanded to a whole spectrum of pathologies of this aortic segment, including dissection and traumatic ruptures. Surgical treatment of degenerative aneurysms of the descending aorta has a very high mortality and morbidity, so TEVAR leads to significantly improved outcomes for these patients. This is the reason the University Hospital Centre (UHC) Zagreb introduced the use of this procedure in the Department of Cardiovascular Diseases. Despite difficulties, TEVAR was used to treat the most at-risk patients with aneurysms, type B dissection, and traumatic rupture of the descending aorta. Long-term results of the treatment of 24 patients were acceptable, with a mortality of 8.3% and common complications (type I and II endoleaks in 12.5%; infection in 4.1%; proximal dissection propagation in 4.1%) which were re- solved with an additional intervention or surgical procedure. In the future we expect much more rapid development of this method in UHC Zagreb and in Croatia in general once the Croatian Health Insurance Fund has arranged financing for the device

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

Repaired Tetralogy of Fallot in Adults – Case Report and a Short Literature Review.

Bolesnici s korigiranom Fallotovom tetralogijom (ToF), zbog razvoja mogućih komplikacija upravo u odrasloj dobi, trebaju se redovito kontrolirati u tercijarnom centru za prirođene bolesti srca (PBS) u odraslih. U slučaju stabilnog tijeka bolesti kontrole su najčešće indicirane u jednogodišnjem intervalu. Važno je, osim detaljne anamneze o eventualnoj pojavi simptoma, uvijek tražiti potencijalne komplikacije. Ako je riječ o značajnoj pulmonalnoj regurgitaciji (PR), što je najčešća kasna komplikaci- ja korigirane ToF, potrebno ju je kvanti cirati te pratiti promjenu u veličini i funkciji desne klijetke (DK). Važno je u donošenju odluke o reoperaciji na pulmonalnoj valvuli (PV) ne čekati predugo radi očuvanja funkcije DK, ali i imati u vidu bržu progresiju disfunkcije proteze ako se ugradi u mlađoj dobi. Budući da su ovakvi bolesnici u jednoj od najrizičnijih skupina unutar populacije odraslih s prirođenim bole- stima srca za pojavu iznenadne srčane smrti, potrebno je njihov rizik strati cirati za sada dostupnim metodama i razmotriti postoji li indikacija za implantaciju kardioverter-defibrilatora. Osim navedenih, potrebno je pri svakoj kontroli tragati i za svim ostalim mogućim komplikacijama radi što boljeg i sveobuhvatnijeg praćenja i liječenja ovih bolesnika.Patients with repaired tetralogy of Fallot (ToF), due to the development of complications in adulthood, need to attend regular follow-up at a tertiary adult congenital heart disease (ACHD) center. If disease progression is stable, the follow-up visits are most often indicated on a yearly basis. In addi- tion to a detailed history of symptoms development, it is important to always look for possible compli- cations. In case of a signi cant pulmonary regurgitation (PR), which is the most frequent late compli- cation of repaired ToF, it needs to be quanti ed, and changes in right ventricular (RV) size and function need to be monitored. It is important not to postpone the re-intervention on the pulmonary valve (PV) for too long in order to preserve the RV function. It is also important to bear in mind the possible faster progression of prosthesis dysfunction if the operation was done at an earlier age. Since repaired ToF patients belong to the group of ACHD conditions with the highest risk of sudden cardiac death (SCD), it is necessary to stratify their risk by using currently available methods and to consider whether there are any indications for the implantation of a cardioverter de brillator. In addition to the above, during each follow-up it is necessary to search for all other possible complications in order to better and more comprehensively monitor and treat these patients

Mycoplasma pneumoniae associated opsoclonus-myoclonus syndrome in three cases

Opsoclonus-myoclonus syndrome (OMS) is a rare acquired movement disorder occurring in all age groups, predominantly in infants. Although the exact pathogenesis is still undefined, there is strong evidence for a paraneoplastic or parainfectious immune process resulting in central nervous system dysfunction. Mycoplasma pneumoniae has been implicated in a number of immune-mediated neurologic diseases [28]. However, the association of M. pneumoniae and opsoclonus-myoclonus-ataxia syndrome is not well established so far. We present three cases with opsoclonus-myoclonus-ataxia syndrome in adolescents following an infection with M. pneumoniae. Monophasic disease course and full recovery correspond to the favorable prognosis known from parainfectious cases in young adults. This should affect therapeutic consideration. OMS should be added to the spectrum of M. pneumoniae-associated neurologic complications. Nevertheless, neuroblastoma has to be ruled out in all cases of OM

Učestalost anatomskih varijacija radijalne arterije u bolesnika podvrgnutih transradijalnoj kateterizaciji srca

Over the last ten years, transradial cardiac catheterization has been increasingly applied, primarily because of its lower incidence of complications compared to the femoral approach. However, one of the greatest flaws of the transradial approach is a relatively high incidence of catheterization failure (1%-5%). Anatomic variations of radial artery are ranked second among the reasons for this. Previous studies have not provided unambiguous data on the frequency of these anomalies. It was therefore the aim of this study to determine the frequency of anatomic variations using routine angiographies of radial artery during left heart catheterization. This was a retrospective study involving examination of 602 images of routine angiographies of radial artery performed during cardiac catheterization. The frequency of anatomic variations of radial artery was 8.8%, exclusive of tortuosities with a frequency of 12.7%. The most frequent anatomic variation was the high origin of radial artery, found in 31 (5.1%) subjects. Radioulnar loops, being one of the potential contraindications for the procedure, were reported in 2% of cases. Regression analysis revealed that age (p<0.001), female sex (p=0.015) and high origin (p=0.034) considerably contributed to the development of tortuosity. The results indicated the incidence of tortuosity to increase linearly with age. Although it is not a contraindication for continuing with the procedure, we recommend that elderly patients have angiography of radial artery performed at the beginning of the procedure due to the higher frequency of tortuosity.Posljednjih desetak godina kateterizacija srca transradijalnim pristupom sve se više koristi prvenstveno zbog rjeđih i lakših mogućih komplikacija u odnosu na femoralni pristup. Ipak, jedan od većih nedostataka transradijalnog pristupa je relativno visoka učestalost neuspjeha kateterizacije (od 1% do 5%). Na drugom mjestu uzroka iste nalaze se anatomske varijacije radijalne arterije (RA). Dosadašnji radovi nisu dali jednoznačne podatke o učestalosti ovih anomalija, stoga je cilj ovoga istraživanja bio odrediti učestalost anatomskih varijacija pomoću rutinskih angiografija RA prilikom PTCA/PCI. Radi se o retrospektivnoj studiji u kojoj su pregledane 602 snimke rutinskih angiografija RA učinjene prilikom kateterizacije srca. Učestalost anatomskih varijacija RA iznosila je 8,8%, ne uključujući tortuozitete učestalost kojih iznosi 12,7%. Najčešća anatomska varijacija je bila visoko polazište RA u ukupno 31 (5,1%) ispitanika. Radioulnarne petlje kao jedna od potencijalnih kontraindikacija za zahvat pojavile su se u 2% slučajeva. Regresijska analiza je pokazala da godine starosti (p<0,001), ženski spol (p=0,015) i visoko izlazište (p=0,034) značajno doprinose razvoju tortuoziteta. Rezultati ukazuju na to da se učestalost tortuoziteta s godinama linearno povećava. Iako isti nisu kontraindikacija za nastavak zahvata, preporučamo da se zbog veće učestalosti tortuoziteta kod starijih bolesnika na početku zahvata učini angiografija RA s ciljem izbjegavanja potencijalnih komplikacija zahvata

Drug-eluting balloons in patients with non-ST elevation acute coronary syndrome

AbstractBackgroundWe compared efficacy of bare-metal stent (BMS) and drug-eluting balloon (DEB) combination vs BMS alone, in patients with non-ST elevation acute coronary syndrome treated with percutaneous coronary intervention (PCI).MethodsPatients with non-ST elevation myocardial infarction (NSTEMI) or unstable angina (UA) were randomized to BMS only or BMS+DEB group. Angiographic follow-up was performed after 6 months. The primary endpoints were binary in-stent restenosis (ISR) and late lumen loss (LLL) and the secondary endpoints were target lesion revascularization (TLR), stent thrombosis (ST), and new acute coronary syndrome (ACS).ResultsA total of 85 patients were enrolled, 44 (BMS) and 41 (BMS+DEB). The median age was 67 (36–84) years and 68 (80%) were male. Fifty-two patients (61.2%) had NSTEMI and 33 patients (38.8%) UA. There was no difference in patient demographics, risk factors, and clinical characteristics, except for more smokers in the BMS+DEB group 18/41 (43.9%) vs 9/44 (20.5%). At follow-up, no significant difference in binary ISR was found; p=0.593, but LLL was significantly lower in the BMS+DEB group 0.68 (0.00–2.15) mm vs 0.22 (0.00–2.35) mm; p=0.002. The difference in major adverse cardiac events (MACE) rate combining TLR, ST, and ACS, between the groups was also non-significant, 29.5% (BMS) vs 24.4% (BMS+DEB); p=0.835. One patient had a subacute ST (BMS+DEB) due to clopidogrel resistance.ConclusionPatients treated with BMS+DEB combination for non-ST elevation acute coronary syndrome had significantly less LLL in comparison to patients treated with BMS alone but without an impact on patient clinical outcomes