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Súdny lekár sa častokrát stretáva s prípadmi náhleho a neočakávaného úmrtia v zdravotníckom zariadení, u ktorých nie sú známe anamnestické údaje o ochoreniach, prípadne tieto údaje sú nedostatočné. Pitva často odhalí nálezy, ktoré sú neobvyklé. Medzi takéto raritné nálezy patria aj rôzne vývinové chyby kardiovaskulárneho systému. Autori prezentujú pitevný nález u 46-ročného muža s truncus arteriosus communis bez chirurgickej intervencie, ktorý zomrel krátko po prevoze do nemocnice za známok kardiorespiračného zlyhania. Truncus arteriosus communis je zriedkavá vrodená kardiovaskulárna anomália, pri ktorej výtoková časť pravej aj ľavej komory ústi priamo do spoločného arteriálneho kmeňa s jednou súpravou chlopní, ktorý obstaráva koronárnu, pľúcnu a systémovú cirkuláciu. Táto zriedkavá anomália je výsledkom zlyhania septácie primitívneho arteriálneho trunku počas embryonálneho života. Jednotlivé typy sú definované na základe miesta odstupu pľúcnych tepien z arteriálneho kmeňa. Celosvetovo predstavuje asi 1-2 % všetkých vrodených vývinových chýb srdca. Incidencia je 5-15 prípadov na 100 000 živonarodených detí. Ide o malformáciu, ktorá bez chirurgickej liečby má veľmi zlú prognózu. Bez liečby je táto kardiovaskulárna anomália zvyčajne fatálna a len asi 15 % jedincov prežije 1 rok života. Celkom výnimočne sa môžu títo jedinci dožiť vyššieho veku aj bez chirurgickej intervencie ako to bolo aj v prezentovanom prípade. Operačné riešenie truncus arteriosus communis nebolo vykonané pre technickú nedostupnosť na Slovensku v čase diagnostikovania anomálie u tohto muža. Kľúčové slová: truncus arteriosus communis – srdcové zlyhanie – pitevný nález

Truncus arteriosus communis is an uncommon congenital cardiovascular malformation characterized by a single arterial trunk that arises from the base of the heart and gives rise to the coronary, pulmonary and systemic arteries. The prognosis in truncus arteriosus is very poor without surgical correction. The median age at death without surgery ranges from 2 weeks to 3 months, with 85 % mortality by age 1 year. The authors report the autopsy findings of a 46 year old man with truncus arteriosus communis without surgical intervention who died at the hospital shortly after admission. Keywords: truncus arteriosus communis – heart failure – autopsy findings

MeSH
aorta thoracica * abnormality MeSH
lidé středního věku MeSH
lidé MeSH
pitva MeSH
srdeční selhání etiologie MeSH
truncus arteriosus persistens * patologie MeSH
vrozené srdeční vady patologie MeSH
Check Tag
lidé středního věku MeSH
lidé MeSH
mužské pohlaví MeSH
Publikační typ
kazuistiky MeSH

Článek online

Long-term results after correction of persistent truncus arteriosus in 83 patients

European journal of cardio-thoracic surgery. 2010 ; 37 (6) : 1278-1284. [pub] 20100204

Eur J Cardiothorac Surg
ISSN 1873-734X
Medvik
Zdroj

OBJECTIVE: This study aims to analyse long-term results after correction of truncus arteriosus in all patients operated in one institution over 28 years. METHODS: Between 1981 and 2009, 83 patients, median age 54 days, underwent repair of truncus arteriosus. Interrupted aortic arch was present in 14 (17%), severe truncal valve insufficiency in 10 (12%) and non-confluent pulmonary arteries in five (6%) patients. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 80, and other methods in three patients. At the same time, correction of interrupted aortic arch was done in 14 and truncal valve repair in eight patients. Survivors were repeatedly examined echocardiographically for assessment of residual heart lesions. RESULTS: The early mortality was 19 (23%). Out of 35 patients operated between 1981 and 1996, 17 (46%) died, and out of 48 patients operated between 1997 and 2009, two (4%) died. Operation before 1997 (p=0.001) and aortic cross-clamping time >90min (p=0.009) were found to be risk factors of death. Eight (10%) patients died late, a median of 68 days after surgery. Fifty-seven (69%) patients were followed for 10.9 + or - 6.7 years. Three (4%) patients were lost. Twenty-five (30%) patients are alive with their original conduit 7.5 + or - 5.2 years after correction. Twenty-eight patients required 41 re-operations for conduit dysfunction with first replacement at mean 5.8 + or - 4.1 (range 0.1-14.1 years) years after correction. Nine (11%) patients required 12 truncal valve replacements. Eleven (13%) patients required balloon dilatation or stent for conduit obstruction, pulmonary branch stenosis, aortic arch obstruction or stenosis of vena cava. Recent clinical examination was undertaken in 53 (64%) patients and 49 (59% or 77% of early survivors) are in good/very good condition. CONCLUSIONS: Truncus arteriosus remains a challenging heart disease. With growing experience, the early mortality decreased to 4%, but numerous re-interventions for conduit obstruction, pulmonary branch stenosis and truncal valve insufficiency are required. Surgery before 1997 and prolonged cross-clamping were risk factors of death. Pulmonary homografts had the best re-intervention-free survival. Statistically, however, the difference between conduits was not significant. Dysplastic valve and truncal valve insufficiency represent risk factors presenting the need for truncal valve replacement.

MeSH
aorta thoracica abnormality chirurgie MeSH
cévy - implantace protéz metody MeSH
chirurgická náhrada chlopně metody MeSH
dítě MeSH
epidemiologické metody MeSH
kojenec MeSH
lidé MeSH
mladiství MeSH
mnohočetné abnormality chirurgie MeSH
novorozenec MeSH
předškolní dítě MeSH
reoperace metody MeSH
selhání protézy MeSH
transplantace heterologní MeSH
truncus arteriosus persistens chirurgie ultrasonografie MeSH
výsledek terapie MeSH
Check Tag
dítě MeSH
kojenec MeSH
lidé MeSH
mladiství MeSH
novorozenec MeSH
předškolní dítě MeSH
Publikační typ
časopisecké články MeSH
práce podpořená grantem MeSH

Článek online

Repair of persistent truncus arteriosus with interrupted aortic arch

Tláskal, Tomáš, 1950-
Autor Autorita
Hučín, Bohumil, 1934-
Autor Autorita
Kučera, Vladimír, 1941-
Autor Autorita
Vojtovič, Pavel
Autor Autorita
Gebauer, Roman
Autor Autorita
Chaloupecký, Václav, 1950-
Autor Autorita
Škovránek, Jan, 1947-
Autor Autorita

Journal of hypertension. 2005 ; 28 (5) : 736-741.

ISSN 0263-6352
Medvik
Zdroj

OBJECTIVE: The aim of our study was to analyse experience with repair of truncus arteriosus with interrupted aortic arch. METHODS: Between 1993 and 2004, eight consecutive patients underwent repair of truncus arteriosus with interrupted aortic arch. The median age was 6.5 days (range 1-85 days) and median weight was 3.2 kg (range 2.6-4.8 kg). Five patients had type A and 3 patients had type B aortic arch interruption. The repair was performed in deep hypothermia with circulatory arrest in 4 patients and isolated selective low-flow perfusion of the head and the heart in the last 4 patients. The repair consisted in aortic arch reconstruction by direct anastomosis between descending and ascending aorta, closure of ventricular septal defect and reconstruction of the right ventricular to pulmonary artery continuity using a valved conduit. RESULTS: One (12.5%) patient died from sepsis and hepato-renal failure 18 days after surgery. Seven (87.5%) patients were followed up for 2.0-11.7 years (median 2.6 years). No patient died after the discharge from hospital. In 4 patients 1-3 reinterventions were required 0.6-10.0 years after repair. Reoperations were performed for conduit obstruction in 2 patients, aortic regurgitation in 2 patients, right pulmonary artery stenosis in 2 patients and airway obstruction in 1 patient. In 2 patients concommitant aortic valve and conduit replacement was required. Balloon angioplasty for aortic arch obstruction was necessary in 1 patient, and for bilateral pulmonary branch stenosis in 1 patient. Five (28.6%) surviving patients are in NYHA class I and 2 (71.4%) patients are in NYHA class II. CONCLUSIONS: Primary repair of persistent truncus arteriosus with interrupted aortic arch can be done with low mortality and good mid-term results. Aortic arch reconstruction in isolated low-flow perfusion of the head and the heart influences favourably the postoperative recovery. The main postoperative problems are associated with conduit obstruction and aortic insufficiency.