We report a very rare case of severe pulmonary arterial hypertension in a patient with neurofibromatosis type 1, and discuss the pathology, pathogenesis, current pulmonary hypertension classification system, and outcomes of pulmonary arterial hypertension in patients with neurofibromatosis type 1.

IntroductionAtrial septal defects (ASDs) are one of the most common congenital malformations in adults. Correction of ASDs in advanced age remains controversial, even though beneficial effects in this patient group were found in recent studies. In older patients, less invasive transcatheter closure of ASDs has been recommended.AimThe aim of this study was to analyze our advanced age ASD surgical cohort: early and late results.Material and methodsRetrospective analysis of 32 patients operated on at an age of ≥ 60 years (i.e. age 66.13 ± 4.8, range from 60 to 78) in our center between 2001 and 2011 was carried out. We reviewed our experience of surgical ASD closure in elderly patients over a 10-year period to assess the effects of this type of treatment on early postoperative and long-term survival, early and late complications, preoperative and postoperative clinical status (New York Heart Association [NYHA] functional class), pulmonary hypertension (PH) and atrial arrhythmias. The patients were divided into two groups according to age.ResultsThe frequency of comorbidities was lower in younger age group patients (11 [61%] vs. 13 [93%], p < 0.05). Atrial fibrillation/flutter was found in 21 (66%) of all patients. Late postoperative mortality was higher in the older patient group (3 [21%] vs. 1 [5.6%]). Despite this, we observed significant improvement of symptoms and functional ability in the older population after surgical ASD closure (group I, n = 10 [56%] vs. group II, n = 12 [86%]).ConclusionsSurgical correction of clinically significant ASD is effective even in older patients with comorbidities.

Pregnancy in patients with Eisenmengers syndrome is associated with high maternal and fetal mortality rates and effective contraception or termination of pregnancy is strongly recommended. When these patients decide to begin or continue a pregnancy against advice a coordinated multi-specialist care at a center for congenital heart disease is mandatory. So far, experience with this demanding group of patients is limited. This report reviews the outcome of 30 pregnancies in 10 patients from a group of 35 women with Eisenmengers syndrome who have been treated at the University Hospital in Vilnius, Lithuania, between 1967 and 2003. The most frequent underlying lesion was a ventricular septal defect in 5 patients, their mean age was 26.2 +/- 5,4 years and they were in functional class II or III (9/1) at the beginning of the pregnancy. In this group 13 pregnancies were terminated (43 %), 4 spontaneous abortions (13 %) occurred at less than 21 weeks of pregnancy and one stillbirth at 23 weeks of gestation. Twelve children were born alive, 10 were premature and 2 term deliveries. One maternal death occurred on the third day following an emergency delivery in week 36 and another patient decompensated immediately following delivery, but could be stabilized with intensive care therapy. During long-term follow-up all of these 9 patients were alive at 10/2003 despite a significant deterioration in functional class. These data are comparable to previously published observations in pregnant women with Eisenmengers syndrome.

TikslasÁvertinti ligoniø, serganèiø Ebðteino anomalija, ðirdies ritmo ir laidumo sutrikimus iki operacijos ir pooperaciniu laikotarpiu. MetodaiRetrospektyviai iðanalizuoti 32 ligoniø, serganèiø Ebðteino anomalija, duomenys, vertinant pavirðinae 12 derivacijø EKG ir EKG monitoravimo 24 val. "Holter" monitoriumi duomenis. RezultataiLigoniø amþius -nuo 1 iki 47 metø (vidurkis 16,5 m.). Prieð operacijà daþniausiai buvo sinusinis ritmas -96% ligoniø, 6% ligoniø -sinusinë tachikardija. WPW sindromas diagnozuotas 12,5% atvejø. Iki operacijos daþniausiai buvo intraskilvelinio laidumo sutrikimai: dalinë deðinës Hiso pluoðto kojytës blokada -34% ligoniø, o visa deðinës Hiso pluoðto kojytës blokada -9% ligoniø.Ávairaus laipsnio atrioventrikulinë blokada nustatyta 15%, prieðirdþiø virpëjimas -9% ligoniø. Po operacijos skilvelinës ekstrasistolës buvo 69% ligoniø, o supraventrikulinës -46% ligoniø. Ávairaus laipsnio AV blokada -30% ligoniø. SMS diagnozuotas 23% ligoniø. Po operacijos "pjûvio" tachikardija diagnozuota 9% ligoniø. IðvadosLigoniams, sergantiems Ebðteino anomalija, bûdingi ávairûs ðirdies ritmo ir laidumo sutrikimai tiek prieð ydos korekcijà, tiek po operacijos. Iki operacijos daþnesni yra intraskilvelinio laidumo sutrikimai, supraventrikuliniai ritmo sutrikimai ir WPW sindromo nulemta paroksizminë tachikardija, reèiau skilvelinë paroksizminë tachikardija. Po chirurginës korekcijos daþnesni skilveliniai ritmo sutrikimai: ekstrasistolija, net skilvelinë paroksizminë tachikardija, taèiau daþnai iðlieka ir supraventrikuliniai ritmo ir laidumo sutrikimai -"pjûvio" tachikardijos, prieðirdþiø virpëjimas, plazdëjimas, sinusinio mazgo disfunkcija ir AV blokada. Dël minëtø ritmo sutrikimø ligoniai, sergantys Ebðteino anomalija, turëtø bûti visà gyvenimà nuolat stebimi kardiologø.Pagrindiniai þodþiai: Ebðteino anomalija, ðirdies ritmo ir laidumo sutrikimai

Lina Gumbienė1, Vytautas Juknevičius2 , Valdas Bilkis1 , Virgilijus Tarutis1 , Diana Zakaraitė1 , Rimantas Karalius1 , Giedrė Nogienė1 , Alicija Dranenkienė1 1 Vilniaus universiteto Širdies ir kraujagyslių ligų klinika, Santariškių g. 2, LT-08661 VilniusEl. paštas: Lina.Gumbiene@santa.lt2 Vilniaus universitetas Medicinos fakultetas Tikslas: Palyginti prieširdžių pertvaros defekto chirurginio ir perkateterinio gydymo metodų ankstyvuosius rezultatus ir komplikacijas. Ligoniai ir metodai: Retrospektyviai tirti vyresni nei 13 metų amžiaus ligoniai, kuriems 2006–2009 m. mūsų ligoninėje buvo gydytas antrinis prieširdžių pertvaros defektas. Rezultatai: 62 ligoniams atlikta chirurginė defekto korekcija (A grupė), 35 ligoniams defektas buvo panaikintas perkateteriniu būdu (B grupė). Vidutinis A grupės ligonių amžius buvo 36,1 metų (nuo 13 iki 71), B grupės – 40,51 metų (nuo 14 ik 72). Viena A grupės ligonė mirė po operacijos išsivysčius infekcinei komplikacijai ir dauginiam organų nepakankamumui. Vienai B grupės ligonei defekto panaikinti nepavyko, dar vienai pacientei po procedūros liko nedidelis nuosrūvis tarp prieširdžių. Komplikacijų nepatyrė daugiau B grupės ligonių negu A grupės (atitinkamai 91,4 % ir 59,67 %, p < 0,05). Sunkių komplikacijų išsivystė tik chirurginiu būdu gydytiems ligoniams. B grupės ligonių hospitalizacijos trukmė buvo statistiškai reikšmingai mažesnė negu A grupės (atitinkamai 4,4 ± 2,6 ir 16,7 ± 16,6 paros, p < 0,0001). Išvados: Ligoniams, kuriems prieširdžių pertvaros defektas uždaromas perkateteriniu būdu, išsivysto mažiau ankstyvųjų pooperacinių komplikacijų ir jų hospitalizacijos laikas trumpesnis. Po chirurginės prieširdžių pertvaros defekto korekcijos atsiradusios ankstyvosios pooperacinės komplikacijos sietinos su buvusiu triburio vožtuvo nesandarumu. Reikšminiai žodžiai: įgimtos širdies ydos, prieširdžių pertvaros defektas, chirurgija, perkateterinis uždarymas Comparison of early results and complications in transcatheter and surgical closure of secundum atrial septal defect Lina Gumbienė1, Vytautas Juknevičius2 , Valdas Bilkis1 , Virgilijus Tarutis1 , Diana Zakaraitė1 , Rimantas Karalius1 , Giedrė Nogienė1 , Alicija Dranenkienė1 1Vilnius University, Clinic of Cardiovascular Diseases, Santariškių str. 2, LT-08661 Vilnius, LithuaniaE-mail: Lina.Gumbiene@santa.lt2Vilnius University Faculty of Medicine Objective: To compare early results and complications in transcatheter and surgical closure of the secundum atrial septal defect (ASD). Patients and methods: rectal prolapse, rectal cancer, faecal incontinence, Altemeyer operation Results: Sixty-two patients underwent surgical correction (Group A), 35 patients underwent transcatheter ASD closure with Amplatzer septal occluder (Group B). The mean age in Group A was 36.14 (13–71) years, in Group B 40,51 (14–72) years. One patient in Group A died because of postoperative infection, heart and renal failure. In Group B, the procedure was unsuccessful in one patent, and residual shunt was found in one patient. The percentage of patients without complications was higher in Group B (91.4% versus 59.7% in Group A, p < 0.05). Severe complications were diagnosed only in the surgical (A) group. The mean length of hospital stay was shorter in Group B (4.4 ± 2.64 days) compared with Group A (16.69 ± 16.55 days, p < 0.0001). Conclusions: The lower rate of early complications and a shorter hospital stay were observed in the transcatheter atrial septal defect closure group. More frequent complications after surgery could have been caused by the insufficiency of the tricuspid valve. Key words: congenital heart disease, atrial septal defect, surgery, transcatheter closure