Sexuality and Reproductive Health in Women With Congenital
Heart Disease
Matthäus Vigl, MDa, Mathias Kaemmerer, Cand. med.b, Eva Niggemeyer, MAa, Nicole Nagdyman, MDc, Vanadin Seifert-Klauss, MDd, Vasiliki Trigas, MDb, Ulrike Bauer, MDa, Karl-Theo M. Schneider, MDd, Felix Berger, MDc, John Hess, MDb, and Harald Kaemmerer, MDb,* The different biopsychosocial periods in a woman’s life are all interactively associated with
the cardiovascular system. The present study was designed to address questions related to
sexuality and reproductive health in a large cohort of women with congenital heart disease.
Overall, 536 women (median age 29 years, range 18 to 75) completed a questionnaire during
their visit at 2 tertiary care centers for congenital heart disease. Patients were categorized
according to their functional class and according to the degree of severity of the underlying
heart defect. The median age at menarche was significantly delayed in patients with
functional class III-IV and in women with complex or cyanotic anomalies. More than 1/4
of the women (29%) had at least once sought medical advice for menstrual discomforts,
and the proportion was significantly increased for those in the worst functional class (49%,
p <0.001) and for patients with a cyanotic heart defect (43%, p
؍ 0.03). Overall, 9%
reported increased or altered symptoms related to their heart defect during sexual activity.
This proportion increased significantly with worsening functional class (6%, 11%, and
26% in functional class I, II, and III-IV, respectively; p
؍ 0.001), increased severity
(5%, 8%, and 17% for simple, moderate, and severe heart defects, respectively; p
0.005), and in women with cyanosis (8% and 28% in acyanotic and cyanotic patients, respec-
tively; p <0.001). In conclusion, to ensure high-quality care for this demanding and growing
patient population, physicians must be aware that issues related to the entire reproductive cycle
should be considered when counseling these patients.

2010 Elsevier Inc. All rights reserved.
(Am J Cardiol 2010;105:538 –541)
The different biopsychosocial periods in a woman’s life, The present study was designed to address questions related including menarche, sexuality, pregnancy, and menopause, to sexuality and reproductive health in a large cohort of are all interactively associated with the cardiovascular sys- women with CHD, with the aim of providing an overview of Nevertheless, contemporary data regarding the sexual the actual situation and generate a basis for future prospec- and reproductive health of women with congenital heart disease (CHD) are scarce. Most studies have been con-cerned with pregnancy-related health and only a few have explored sexuality and other aspects of reproduc-tive health in this patient Currently, most During a 12-month period, 536 consecutive adult female recommendations and patient information for women with patients with CHD, who were seen at the outpatient clinic of CHD have been based solely on expert knowledge because 2 tertiary care centers for adults with CHD (Deutsches existing scientific evidence has not provided enough infor- Herzzentrum München and Deutsches Herzzentrum Berlin) mation to design adequate individual counseling strategies.
were included. The inclusion criteria were confirmed CHD,age Ն18 years, and written consent. The lack of cognitivecompetency to understand and complete the questionnaire Competence Network for Congenital Heart Defects, Deutsches Herz- The women were required to complete a questionnaire zentrum Berlin, Berlin, Germany; bDepartment of Pediatric Cardiology andCongenital Heart Disease, Deutsches Herzzentrum München, Munich, designed for self-administration. This questionnaire covered Germany; cDepartment of Congenital Heart Defects and Pediatric Cardi- different aspects, including demographics and sexual and ology, Deutsches Herzzentrum Berlin, Berlin, Germany; and dFrauen- reproductive health issues. A separate questionnaire was klinik, Technische Universität München, Munich, Germany. Manuscript compiled by the treating physician, including cardiac and received August 18, 2009; revised manuscript received and accepted Oc- noncardiac diagnoses and surgical and pharmacologic treat- ment. The medical records were obtained from all partici- This work was supported by the Kompetenznetz Angeborene Her- pating patients and reviewed, if necessary.
zfehler (Competence Network for Congenital Heart Defects), Berlin, Ger- The medical and surgical records were reviewed for ana- many, funded by the Federal Ministry of Education and Research (BMBF), tomic characteristics before repair and for details of surgical repair and reoperation. Using the medical history and clinical *Corresponding author: Tel: (ϩ49) 89-1218-3006; fax: (ϩ49) 89- assessment findings, the attending physician classified the E-mail address: (H. Kaemmerer).
patients according to 1 of 4 functional This clas- 0002-9149/10/$ – see front matter 2010 Elsevier Inc. All rights reserved.
Congenital Heart Disease/Women’s Reproductive Health and CHD Baseline characteristics of study participants (n ϭ 536) Congenital heart defect diagnoses of study participants (n ϭ 536) * According to American College of Cardiology classification.
† Percentage of those who underwent surgery.
‡ Included patients with a history of thrombosis, pulmonary embolism, Data in parentheses are ranges, unless otherwise noted.
* Double outlet right ventricle (n ϭ 10), aortic regurgitation (congenital, n ϭ 7), subaortic stenosis (n ϭ 6), cardiomyopathy (congenital, n ϭ 6),partial anomalous pulmonary venous connection (n ϭ 6), aortic anomalies(congenital, n ϭ 5), mitral regurgitation (congenital, n ϭ 5), pulmonary sification was specially developed for adults with CHD and atresia (n ϭ 4), tricuspid regurgitation (congenital, n ϭ 4), arrhythmias is similar to the New York Heart Association classification (congenital forms, n ϭ 3), cor triatriatum (n ϭ 2), double-chamber right for patients with heart failure. Only 2 patients were in ventricle (n ϭ 2), Bland-White-Garland syndrome (n ϭ 2), supravalvular functional class IV. To allow statistical analysis, they were aortic stenosis (n ϭ 2), aortopulmonary window (n ϭ 1), arrhythmogenic grouped with the 39 patients with functional class III, form- right ventricular dysplasia (n ϭ 1), interrupted aortic arch (n ϭ 1), and ing functional class III-IV, a group of symptomatic patients with restrictions even in performing daily activities.
For additional analysis, the patients were assigned a severity code. This codification followed the recommenda-tion of the American College of Cardiology and facilitated from answering some of the questions. This same reason the allocation of patients to 1 of 3 degrees of severity made on-site control of the completeness of the compiled (simple, moderate, or severe) depending on the underlying questionnaires impossible to guarantee maximum confiden- tiality. Therefore, relative percentages of the answers were The data were analyzed using the Statistical Package for calculated, and the number of missing information was Social Sciences, version 12.0 (SPSS, Chicago, Illinois). The always reported for the respective questions.
descriptive statistics of continuous variables were calculated The institutional ethics committees of the 2 participating as the mean Ϯ SD or as the median, in the case of a non-normal distribution. Nominal variables are expressed asfrequencies and percentages. Chi-square tests were used to detect differences in the nominal variables between groups,and, if Ͼ20% of the expected counts were Ͻ5, Fisher’s In the 12-month recruitment period, 536 adult women exact test was applied. Differences between continuous with CHD and a median age of 29 years (range 18 to 75) variables were measured with unpaired t tests and with the were included in the present study. Some baseline charac- Mann-Whitney U test when the data did not meet the as- teristics and the diagnoses of the included patients are listed sumption of normal distribution. Odds ratios were calcu- in and The first menarche, as the culmination of lated from 2 ϫ 2 tables and are presented with the 95% a series of physiologic processes of female puberty, oc- confidence intervals. The intimate nature of the questioned curred at a mean age of 13.0 Ϯ 1.6 years (range 9 to 19, information might have prevented some of the participants missing data for 15). Women with more complex heart The American Journal of Cardiology ( Table 3Mean age at menarche and proportion of women with menstrual discomfort and cardiac complaints during menstruation (n ϭ 536) * Only menstrual discomfort for which medical advice was sought was considered.
Table 4Mean age at first sexual intercourse and proportion of women with cardiac complaints during sexual activity (n ϭ 536) anomalies and women with a cyanotic heart defect were provided their age at their first sexual intercourse. The mean significantly older at their first menarche age was 17.7 Ϯ 2.5 years (range 12 to 32). Of these women, Of 486 patients (missing data for 50), 139 (29%) had at 15% (n ϭ 69) had had their first sexual intercourse at Յ15 least once sought medical advice for menstrual discomforts.
The most frequent complaint was menstrual pain (23%), Overall, 9% (missing data for 34) reported increased or followed by cycle irregularities (17%), severe menstrual altered symptoms related to their heart defect during sexual bleeding (14%), and amenorrhea (5%). Women taking an- activity. This proportion increased significantly with wors- tiplatelet or antithrombotic medication did not differ in the ening functional class, increased degree of severity, and reported frequency of menstrual irregularities, with the ex- cyanosis. Of the complaints during sexual activity, dyspnea, ception of women taking anticoagulant medication, for perceived arrhythmia, increased fatigue, and syncope were whom an almost threefold increased risk of menorrhagia was found (odds ratio 2.8, 95% confidence interval 1.6 to5.1). Overall, the proportion of women complaining of Discussion
menstrual discomfort was significantly greater in the worstfunctional class and in patients with a cyanotic heart defect Our study on the specific sexual and reproductive health- related problems has presented data from Ͼ500 sexually Furthermore, 8% of the participants (missing data for 29) mature women with nearly all types of congenital cardiac complained of increased or modified cardiac complaints anomalies (native, after surgery, or interventional treatment) during menstruation, with the rates particularly high among and across all age groups. According to our data, the median women in the worst functional class and among women age of the participating women at menarche was 13.0 years.
with cyanosis. The proportion of women with increased This is close to the 12.8 years reported for the general cardiac complaints during menstruation did not differ sig- German but younger than the 13.3 to 13.4 years nificantly among the degrees of severity. Increased fatigue, found in other studies of patients with Menarche dyspnea, chest pain, tachycardia, impression of arrhythmia, was significantly delayed in women with complex or cya- and dizziness were most frequently cited Of all the patients, 6% (n ϭ 35) reported never having Furthermore, Ͼ1/4 of the surveyed women had at least had sexual intercourse. Of the remaining 501 women, 450 once experienced one or more types of menstrual disorders.
Congenital Heart Disease/Women’s Reproductive Health and CHD Because we had to rely on subjectively reported data, we than that seen in either community-based hospitals or car- included only discomfort for which at least once medical advice had been sought in the analysis. An increased risk ofmenstrual disorders and menstrual irregularities has beenpreviously reported. In those studies, the proportion of men- Acknowledgment: The authors thank the participating
strual problems was significantly associated with the pres- women for giving insights into these intimate parts of their ence of cyanosis, the number of surgical interventions, and life, the staff of the outpatients’ clinic for adults with CHD the severity of the This is in line with our findings at the German Heart Center Munich and Berlin for their in which the prevalence of menstrual disturbances was sig- contribution in motivating and including the patients, and nificantly increased in the worst functional classes and in Leona Bauer and Markus Paulick for organizational help In addition, cardiac complaints related to the heart defect seem to deteriorate during the menstrual period in some 1. Somerville J. Congenital heart disease in the adolescent. Arch Dis affected women. Again, this observation was more pro- nounced in the worst functional classes and in cyanotic 2. Drenthen W, Pieper PG, Roos-Hesselink JW, van Lottum WA, Voors AA, Mulder BJ, van Dijk AP, Vliegen HW, Yap SC, Moons P, Ebels patients. The causal biologic pathway between the cardiac T, van Veldhuisen DJ. Outcome of pregnancy in women with congen- anomaly and ovarian function has never been analyzed in ital heart disease: a literature review. J Am Coll Cardiol 2007;49: depth and no prospective data are available. It has been hypothesized that these abnormal menstrual patterns repre- 3. Drenthen W, Hoendermis ES, Moons P, Heida KY, Roos-Hesselink sent recurrent anovulatory cycles, dysfunction of the hypop- JW, Mulder BJ, Van Dijk AP, Vliegen HW, Sollie KM, Berger RM,Lely AT, Canobbio MM, Pieper PG. Menstrual cycle and its disorders ituitary-ovarian axis, or disturbed uterine hemostasis due to in women with congenital heart disease. Congenit Heart Dis 2008;3: cardiac defect itself, repeated surgical intervention during 4. Moons P, Engelfriet P, Kaemmerer H, Meijboom FJ, Oechslin E, childhood might have interfered with the complex physio- Mulder BJ. Delivery of care for adult patients with congenital heart logic processes involved in the ovarian cycle.
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cardiac anomalies and the ovarian cycle, additional studies, 9. Canobbio MM, Rapkin AJ, Perloff JK, Lin A, Child JS. Menstrual including the analysis of hormones and biomarkers, are patterns in women with congenital heart disease. Pediatr Cardiol1995;16:12–15.
10. Seifert-Klauss V, Prechtl E. Dysmenorrhoe. In: Kiechle M, Gerhard I, The median age at the first sexual intercourse of the eds. Medizin Integrativ: Gynäkologie. München: Elsevier Urban und included women was 17.7 years. This is within the age range of 16.5 to 18.5 years for European countries and close 11. Seifert-Klauss V. Zyklusstörungen. In: Kiechle M, Gerhard I, ed.
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lated to the cardiac situation in Ͼ1/4 of women with func- Assessment of the prevalence and impact of anemia on women hos-pitalized for gynecologic conditions associated with heavy uterine tional class III-IV, as well as in the group of cyanotic bleeding. J Reprod Med 2008;53:323–330.
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tional design and the lack of a control group. Furthermore, 16. Bundesministerium für Familie, Senioren, Frauen und Jugend (BFSFJ), the study was performed at 2 tertiary care centers for adults ed. Bericht zur gesundheitlichen Situation von Frauen in Deutschland.
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Capitulo 001.p65

Capítulo XXII Os contrabandistas Dantès não passara um dia sequer a bordo, e já reconhecera o que tinha pelafrente. Sem ter estado na escola do abade Faria, o digno patrão da Jeune-Amelie,este era o nome do barco genovês, sabia quase todas as línguas faladas em tornodeste grande lago que chamam de Mediterrâneo, desde o árabe até o provençal;isto dava a ele, evitando os tradutores

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