Deceased organ donation in Brazil: how can we improve?

METHODS: We retrospectively analyzed the registry data from one organ procurement organization obtained between January 1 and December 31, 2005. RESULTS: Among the 378 potential deceased donors, 182 (48.2%) were lost, mainly due to clinical conditions (27%) or cardiac arrest (19.3%). Of the remaining 196 (51.8%) potential donors, family consent was obtained in 94 cases (48%). Family refusal was higher for potential donors aged between 18 and 59 years (70%). Of the 94 donors, 72 (77%) had their organs harvested. Cardiac arrest before harvesting (56.5%) and positive viral serology (26%) were the main reasons for further losses. The mean donor age was 40 years and 51% were men. Causes of death were cerebral vascular accidents (55.5%), cranium encephalic traumas (29%), and gun shot wounds (8%). The rate of organ donation was 100% for kidneys and livers, 96% for hearts, 86% for pancreatas, 76% for lungs, and 74% for corneas. After assessment of organ viability, 94% of corneas, 91% of kidneys, and 88% of livers were transplanted, but only 52% of pancreata and 42% of hearts. The most frequent causes of discarded organs were age and concomitant donor infection. CONCLUSION: Areas for potential improvements are: (1) earlier identification and adequate maintenance of potential donors; (2) campaigns for organ donation; and (3) careful evaluation of donated organs and selection of a suitable population to increase utilization of expanded criteria organs.

Cardiac malformations in patients with oral-facial-skeletal syndromes: clinical similarities with heterotaxia.

Oral-facial-skeletal (OFS) syndromes include short rib-polydactyly (SRP) and oral-facial-digital (OFD) syndromes. Congenital heart defect (CHD), mainly atrioventricular canal defect (AVCD), is a cardinal finding in the Ellis-van Creveld (EVC) syndrome, but it occurs only occasionally in other SRP and OFD syndromes. The cardiac characteristics of all patients with OFS syndromes evaluated at our hospital from January 1986 to April 1997 were analyzed and compared with published reports. Ten patients with EVC syndrome, one with McKusick-Kaufman syndrome, and one with OFD syndrome type II had AVCD. Eight patients (67%) had a common atrium, eight (67%) a persistent left superior vena cava (LSVC) draining into the left atrium because of an unroofed coronary sinus in five (42%), and left-sided obstructive lesions in three (25%). One patient with EVC syndrome had AVCD, common atrium, double outlet right ventricle, persistent LSVC associated with "asplenia syndrome," visceral heterotaxia, and right isomerism. The combination of CHDs found in the personal series of OFS syndromes suggests pathogenetic similarity with heterotaxia syndromes. Published results also corroborate the association between OFS syndromes and CHDs usually occurring in heterotaxia. Molecular studies could shed light on the genetic mechanisms implicated in the cause of the OFS and heterotaxia syndromes.

Echocardiographic evaluation of left ventricular systolic function in the Down syndrome.

Left ventricular systolic function was evaluated by echo-Doppler in 22 Down syndrome patients without congenital heart disease. Although they had evident left ventricular hyperkinesia, this did not appear to reflect intrinsic abnormalities of myocardial properties but a reduced afterload.

Congenital mitral valve anomalies in transposition of the great arteries.

Seventy-nine specimens with transposition of the great arteries were reviewed. Among them were 45 with ventricular septal defect, including 6 with so-called "posterior aorta transposition". The morphology of the mitral valve was carefully studied. Mitral valve anomalies were found in 24 cases (30%). The anomalies involving the mitral valve were classified into 4 groups according to the different components of the valve: A) specimens with anomalies of the leaflets--2 cases with mitral cleft; B) those with anomalies of the commissures--1 case with a mitral gap; C) those with abnormalities of the chordae tendinae--2 cases of malattachment of the chordae tendinae to the ventricular septum and 2 with bridging chordae between the papillary muscles; and D) those with abnormalities of the papillary muscles--5 cases with a parachute valve, 10 with a "form fruste" of the parachute mitral valve (hypoplasia of one papillary muscle and short chordae tendinae) and 2 with abnormally placed papillary muscles, which reached the pulmonary annulus. In 14 cases, abnormalities of the tricuspid valve were also encountered, including 8 cases with a tricuspid gap and 6 with a malattachment of tricuspid chordae on the border of a ventricular septal defect. It can be concluded that mitral valve anomalies are not uncommon in transposition of the great arteries. This pathology must be carefully evaluated at the time of surgery, particularly in those patients for whom an anatomic correction is considered, for in those cases the left ventricle will be supplying the systemic circulation and therefore, a completely normal mitral valve will be required.(ABSTRACT TRUNCATED AT 250 WORDS)

Unexplained plexogenic pulmonary arteriopathy: an unusual rapidly progressive case in childhood.

A 4-year-old child died because of unexplained plexogenic arteriopathy only seven months after the onset of the symptoms. Cardiac catheterization revealed a primary pulmonary hypertension sensible to nifedipine, but after the initial favourable results the clinical course rapidly went down and the child died from right sided congestive heart failure. The post-mortem pulmonary histology revealed reversible structural abnormalities. It also demonstrates that the vasodilatator drugs are effective only for short time in primary pulmonary hypertension, and moreover that it is even hazardous to generalize when one talks about reversibility of such lesions in each case.

Palliation of cardiac malformations associated with right isomerism (asplenia syndrome) in infancy.

Right isomerism is characterized by the combination of an obstructed pulmonary outflow tract and a total anomalous pulmonary venous connection (TAPVC), which is obstructed in nearly half of the patients. Fourteen patients less than 2 years of age with right isomerism have been seen in our unit. Thirteen of them underwent palliation consisting of a systemic-pulmonary shunt (10 patients) or a combination of shunt and TAPVC repair (3). There were 7 hospital deaths (54%). The presence of obstructed pulmonary venous drainage was the major risk factor in the surgical treatment of these complex cardiac anomalies (p less than .02). Guidelines for the palliative management of right isomerism are suggested.

Complete transposition of the great arteries: visualization of left and right outflow tract obstruction by oblique subcostal two-dimensional echocardiography.

Subcostal oblique 2-dimensional echocardiography was performed in 64 infants younger than 2 years with complete transposition of the great arteries (TGA) (situs solitus, concordant atrioventricular and discordant ventriculoarterial connections). All patients examined before cardiac catheterization had a correct diagnosis by 2-dimensional echocardiography using the subcostal oblique views. Twelve patients had associated left ventricular (LV) outflow tract obstruction and 7 had right ventricular (RV) outflow obstruction. The standard parasternal views failed to diagnose obstruction in 1 patients with LV outflow obstruction and 5 with RV outflow obstruction; the subcostal left oblique cut and long axis of the left ventricle visualized all left-sided obstructions, and right-sided obstructions were correctly displayed in 5 of 7 cases using a combination of left oblique and right oblique cuts. Two-dimensional echocardiographic subcostal oblique views allow an excellent definition of the morphologic characteristics of RV and LV outflow tracts in patients with TGA and improve the diagnosis of the outflow obstruction in these malformations.