Psychosocial functioning of the adult with congenital heart disease: a 20-33 years follow-up.

AIMS: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.     

Rapid and accurate measurement of LV mass by biplane real-time 3D echocardiography in patients with concentric LV hypertrophy: comparison to CMR.

AIMS: To evaluate the accuracy of real-time three-dimensional echocardiography (RT3DE) using a biplane and multiplane method in determining left ventricular (LV) mass compared to cardiac magnetic resonance imaging (CMR). METHODS AND RESULTS: LV mass was measured in 18 adult patients with congenital aortic stenosis using CMR and echocardiography (M-mode, two-dimensional echocardiography (2DE), and RT3DE). RT3DE data were analysed using a biplane and multiplane method. No geometric assumptions were necessary using the multiplane RT3DE method. With regard to biplane or multiplane RT3DE, no tendency of over- or underestimation of LV mass was observed. Pearson's correlation coefficients for RT3DE versus CMR were 0.84 and 0.90 for the biplane and multiplane method, respectively. In addition, the accuracy of both RT3DE methods were comparable (Fisher's R-to-Z transformation: Z = 0.69, P = NS). Finally, off-line analysis using biplane RT3DE was significantly faster than multiplane RT3DE (3.8 +/- 1.2 vs. 7.8 +/- 1.7 minutes, P < 0.001). CONCLUSIONS: Biplane RT3DE provided an accurate estimate of LV mass in patients with concentric left ventricular hypertrophy, which was not improved by multiplane RT3DE. The accuracy and speed of analysis renders biplane RT3DE an attractive tool in daily clinical practice for assessing the degree of LV hypertrophy.     

Development and validation of a spina bifida-specific pediatric quality of life questionnaire: the Spina Bifida Pediatric Questionnaire,SBPQ

Purpose

Based on existing questionnaires and patient interview, a health-related quality of life (HRQoL) questionnaire in spina bifida (SB) children is created and validated, the Spina Bifida Pediatric Questionnaire (SBPQ).

Methods

SB patients from the SB reference centre Ghent University Hospital, Belgium, with mental ability between 6 and 18 years old and their parents were asked to participate in the study, together with a control group.

Results

Thirty-nine patients and parents answered the questionnaire once, 20 patients and their parents the test-retest. Thirty-five controls answered the questionnaire once, 34 controls and their parents the test-retest. The final questionnaire was retained when 3 consecutive patients approved all items. Visual clues were added for children with a mental ability below 10 years of age. The test-retest showed a good to excellent agreement for child self-report in 5 domains (not for social functioning), for parent proxy report in all domains (6), for control self-report in 4 domains (not for domain home) and for control parent proxy report in all domains (5). Internal consistency reliability was good in child self-report and in parent proxy report, except for physical functioning in child self-report. There was parent–child agreement for 4 out of 6 domains. Regarding social and emotional functioning, QoL was rated lower by parents than by children themselves.

Conclusion

A SB HRQoL questionnaire was developed and validated. Because of visual aid, this questionnaire can be used by both young children and adolescents.

     

Problems with Eating and Drinking in Patients with Unilateral Peripheral Facial Paralysis

Patients with facial paralysis not only suffer from asymmetry of the face, but also from problems with eating and drinking. To demonstrate that these patients have many problems with activities such as eating and drinking, we examined 17 outpatients with a unilateral peripheral facial paralysis for the presence of problems in these functions. To collect data, all patients completed a questionnaire concerning the problems they had directly following onset of the paralysis and at the moment of this study. In addition, they were examined to determine the severity of the paralysis, the problems with eating and drinking, their experiences regarding impairments and disabilities, and the compensatory behavior. The result of the study is a portrayal of problems and conscious or unconscious compensations. Furthermore, we concluded that the Sunnybrook Facial Grading System score does not predict the number of problems in eating and drinking, the number of compensations, and the emotional impact. Significant correlations are found only between various scores on Visual Analog Scales and the number of compensatory actions. Patients differ in how they experience the extensive problems due to the facial paralysis and in the extent to which they are successful in adaptation. Consulting a speech therapist for treatment of functional problems is a meaningful adjunct to regular therapy. The treatment of deglutition disorders can be best based upon the detailed information of the questionnaire and the examination of eating, drinking, and compensatory behavior.     

Hemodynamic adaptation to pregnancy in women with structural heart disease

Background

Many women with structural heart disease reach reproductive age and contemplate motherhood. Pregnancy induces and requires major hemodynamic changes. Pregnant women with structural heart disease may have a reduced cardiac reserve. There are no longitudinal data on cardiovascular adaptation throughout pregnancy in women with structural heart disease.

Methods

Thirty-five women with structural heart disease were included in a prospective observational trial. Maternal hemodynamics were assessed before conception, during pregnancy and 6 months postpartum by transthoracic echocardiography. Uteroplacental perfusion was analyzed by obstetric Dopplers. Longitudinal evolution over time was analyzed as well as the long term influence of pregnancy on cardiac function.

Results

Cardiac output (CO), stroke volume (SV), left ventricular mass (LV mass) and E/E′ ratio significantly increased and ejection fraction (EF) and fractional shortening (FS) decreased during pregnancy. There was a statistically significant difference in EF, FS and E/E′ ratio before and after pregnancy.

Conclusions

The characteristic pattern of hemodynamic adaptation to pregnancy is attenuated in women with structural heart disease. The pregnancy related volume load induces progression of diastolic dysfunction. Our data suggest a persistent reduction in systolic and diastolic cardiac functions after pregnancy in women with structural heart disease.     

Mucinous adenocarcinomas: poor prognosis in metastatic colorectal cancer

PurposeMucinous histology of metastatic colorectal cancer (CRC) has been associated with poor prognosis, however this has never been assessed in large well-defined study populations treated with the current used systemic agents. We investigated the prognostic value of mucinous histology in two large phase III studies in metastatic CRC.Patients and methodsThe study population included 1010 metastatic CRC patients who were treated with chemotherapy and targeted therapies in two phase III studies. Patients were classified according to the histology of the primary tumour in mucinous adenocarcinomas (MC) and non-mucinous adenocarcinomas (AC).ResultsPatients with MC (n = 99) were older, had more often a normal serum lactate dehydrogenase (LDH), extrahepatic localisation of metastases, larger primary tumour diameter and a higher T classification compared to patients with AC (n = 911). A deficient mismatch repair system and BRAF mutations were observed in 17% and 22% of patients with MC, compared to 3% and 7% in patients with AC, respectively. Clinical outcome was investigated in both studies separately, showing a worse overall survival (OS), progression free survival and overall response rate in patients with MC compared to patients with AC. Patients with MC received less cycles of treatment compared to AC, but did not suffer from a higher incidence of grade 3/4 toxicity. In multivariate analysis, mucinous histology was as an independent negative prognostic factor for OS, resulting in a combined hazard ratio of 1.78 (95% confidence interval (CI) 1.35–2.35).ConclusionsPatients with metastatic mucinous CRC have distinct clinicopathological features and poor response to chemotherapy and targeted agents. The strong negative prognostic value of MC warrants the use of this pathological feature as a stratification factor for clinical trials in metastatic CRC.     

Outcome of pregnancy in women after pulmonary autograft valve replacement for congenital aortic valve disease

BACKGROUND AND AIM OF THE STUDY: The pulmonary autograft has been recommended as the valve of choice for aortic valve replacement (AVR) in young women contemplating pregnancy. However, current information on maternal and perinatal outcome of pregnancy in women with pulmonary autograft valve replacement is limited. METHODS: Using a nationwide Dutch registry (CONCOR) and a local Belgian tertiary care center database, 17 women (age range: 18 to 45 years) with pulmonary autograft valve replacement were enrolled into the study. Twelve pregnancies were observed among five different women, including one miscarriage and one elective abortion. RESULTS: Clinically significant (non-)cardiac complications were documented in two of 10 completed pregnancies. Complications included: (i) placental abruption necessitating Cesarean delivery at 29 weeks' gestation, further complicated by postpartum hemorrhage; and (ii) preterm premature rupture of the membranes resulting in premature delivery at 29 weeks' gestation with postpartum demise of the immature born child. Two women reported primary female infertility, but both became pregnant after hormonal substitution therapy. Four women reported irregularities of their natural menstrual cycle (menorrhagia, dysmenorrhea, polymenorrhea, oligomenorrhea, or amenorrhea). CONCLUSION: Successful pregnancy in women with pulmonary autograft valve replacement is possible, although serious and clinically significant events occurred during gestation. Infertility and menstrual cycle disorders appear to be more prevalent.