Marfan Syndrome with Aortic Root Disease, Severe Heart Failure and Aortic Dissection- Two Case Reports.

Marfan syndrome is an uncommon inheritable connective tissue disease which affects the cardiovascular system. This paper presents two cases of Marfan Syndrome with predominant aortic root disease that were seen at the Cardiology Clinic of University of Nigeria Teaching Hospital, Enugu, Nigeria. Their biography, clinical features and echocardiography parameters were captured using structured questionnaire. Both were young males in their 4th decade of life, and had advanced aortic root diseases which were complicated by left ventricular failure in both, while one of them had aortic dissection and ischemic stroke. Young adult Nigerians with Marfan syndrome presented with advanced aortic root diseases, heart failure, aortic dissection and stroke.

Electrocardiographic findings in a cross-sectional study of human immunodeficiency virus (HIV) patients in Enugu, south-east Nigeria.

BACKGROUND: Electrocardiographic (ECG) abnormalities are prevalent in subjects with human immunodeficiency virus (HIV) infection. In this study, three groups of subjects were investigated and the prevalence of ECG abnormalities was analysed. METHODS: A cross-sectional study was carried out on adults between November 2010 and November 2011 at the University of Nigeria Teaching Hospital, Enugu, Nigeria. One hundred HIV-infected patients on highly active anti-retroviral therapy (HAART), 100 HIV-infected HAART-naïve patients and 100 HIV-negative controls were recruited. Twelve-lead electrocardiograms were done on all subjects. Data were analysed using the chi-squared, Student's t-, one-way ANOVA and Duncan post hoc tests. RESULTS: Left-axis deviation was seen in 15 (16%) of the HIV-positive subjects on HAART, 10 (13.7%) of the HAART-naïve subjects and eight (21%) of the controls (p = 0.265). Eight (11%) subjects with left ventricular hypertrophy (p < 0.001) and two (2.7%) with ST-segment elevation were found among the HIV-positive HAART-naïve subjects (p = 0.134). Prolonged QTc interval was seen in 17 (18.2%) of the HIV-positive patients on HAART, 12 (16.4%) of the HIV-positive HAART-naïve patients and four (10.5%) of the controls (p = 0.012). CONCLUSION: The prevalence of ECG abnormalities was higher in the HIV-positive patients on HAART (93%) and the HIV-positive HAART-naïve patients (73%) compared to the controls.

Lipid and Some Other Cardiovascular Risk Factors Assessment in a Rural Community in Eastern Nigeria.

BACKGROUND: Continuous re-evaluation of modifiable cardiovascular risk factors (cardiovascular diseases [CVDs]) in developing nations is imperative as it lays foundation for early preventive/intervention measures at grass root level to improve/prevent CVD morbidity and mortality in those nations where health indices still score below the standard. AIM: The aim was to assess CVD risk factors as a continuous re-evaluation of these may underscore the need for early intervention measures at grass root level. SUBJECTS AND METHODS: A total of 257 apparently healthy inhabitants aged 18-85 years were recruited in a rural community in South Eastern Nigeria by convenient sampling. Blood pressure, waist circumference and blood lipid analysis were done procedurally and data analyzed using SPSS 16.0 statistical software. RESULTS: The males were older (59.41 [5.22]) than the females (53.31 [16.90]). 69.2% (133/192) were low level farmers, retirees and dependents. Total cholesterol (TC), low density lipoprotein (LDL), and risk predictive index were higher in females while triglyceride (TG), high density lipoprotein and very LDL (VLDL) were higher in males. The middle aged and elderly respectively had higher TG and VLDL compared to the young. Aside hypertriglyceridemia, all lipid abnormalities were higher in females than males both singly (high TC: 28.9% [35/121] vs. 16.9% [12/71]; high LDL cholesterol: 52.0% [63/121] vs. 31.0% [22/71]) and in combination hypercholesterolemia with hypertriglyceridemia (42.9% [52/121] vs. 36.6% [26/71]). "Multiple risk factors" also occurred more in females with seeming further increase in older age. CONCLUSION: The chances of a female having CVD after menopause seemed to outweigh that of the male. CVD preventive measures should be focused at the primary/community level as a means to curtailing the increasing morbidity and eventual mortality from CVDs.

Development of the roadmap and guidelines for the prevention and management of high blood pressure in Africa: Proceedings of the PASCAR Hypertension Task Force meeting: Nairobi, Kenya, 27 October 2014.

Africa has one of the fastest growing economies in the world. The economic changes are associated with a health transition characterised by a rise in cardiovascular risk factors and complications, which tend to affect the African population at their age of maximum productivity. Recent data from Africa have highlighted the increasing importance of high blood pressure in this region of the world. This condition is largely underdiagnosed and poorly treated, and therefore leads to stroke, renal and heart failure, and death. Henceforth, African countries are taking steps to develop relevant policies and programmes to address the issue of blood pressure and other cardiovascular risk factors in response to a call by the World Health Organisation (WHO) to reduce premature deaths from non-communicable diseases (NCDs) by 25% by the year 2025 (25 × 25). The World Heart Federation (WHF) has developed a roadmap for global implementation of the prevention and management of raised blood pressure using a health system approach to help realise the 25 × 25 goal set by the WHO. As the leading continental organisation of cardiovascular professionals, the Pan-African Society of Cardiology (PASCAR) aims to contextualise the roadmap framework of the WHF to the African continent through the PASCAR Taskforce on Hypertension. The Taskforce held a workshop in Kenya on 27 October 2014 to discuss a process by which effective prevention and control of hypertension in Africa may be achieved. It was agreed that a set of clinical guidelines for the management of hypertension are needed in Africa. The ultimate goal of this work is to develop a roadmap for implementation of the prevention and management of hypertension in Africa under the auspices of the WHF.

Intra-cardiac masses in adults: a review of echocardiogram records at two echocardiographic laboratories in Enugu, South-East Nigeria.

BACKGROUND: Transthoracic echocardiography (TTE) is an excellent initial diagnostic technique used to evaluate and diagnose cardiac masses, even though transoesophageal echocardiography (TEE) provides superior image resolution and better visualization of cardiac masses, especially in patients with suboptimal transthoracic echocardiographic studies. TTE is the clinical procedure of choice for identification of left ventricular thrombi. TTE has greater than 90% sensitivity and greater than 85% specificity for detection of left ventricular thrombi and is probably superior to the sensitivity and specificity of TEE, especially for apical thrombi. AIMS: The study aimed to identify the common types of cardiac masses and their commonest locations in the heart. MATERIALS AND METHODS: We did a retrospective review of our echocardiogram reports from May 2003 to July 2012 to identify the frequency of intra-cardiac masses in adults, as well as the gender distribution and commonest location of these masses. RESULTS: There were 2,814 echo examinations in adults over this period, comprising 1,661 males (59.1%) and 1,153 females (40.9%). Intra-cardiac masses were found in 20 of these patients representing 0.7% of the study population. Thrombi were the commonest masses noted in our study, and there were more masses in the atria than in the ventricles. The left heart chambers also had more masses than the right heart chambers. There was no sex difference in the frequency of cardiac masses. CONCLUSION: Intra-cardiac masses are rare, and transthoracic echocardiography is still valuable in the diagnosis and initial characterization of cardiac masses.

Efficacy of newer versus older antihypertensive drugs in black patients living in sub-Saharan Africa.

To address the epidemic of hypertension in blacks born and living in sub-Saharan Africa, we compared in a randomised clinical trial (NCT01030458) single-pill combinations of old and new antihypertensive drugs in patients (30-69 years) with uncomplicated hypertension (140-179/90-109 mm Hg). After ≥4 weeks off treatment, 183 of 294 screened patients were assigned to once daily bisoprolol/hydrochlorothiazide 5/6.25 mg (n=89; R) or amlodipine/valsartan 5/160 mg (n=94; E) and followed up for 6 months. To control blood pressure (<140/<90 mm Hg), bisoprolol and amlodipine could be doubled (10 mg per day) and α-methyldopa (0.5-2 g per day) added. Sitting blood pressure fell by 19.5/12.0 mm Hg in R patients and by 24.8/13.2 mm Hg in E patients and heart rate decreased by 9.7 beats per minute in R patients with no change in E patients (-0.2 beats per minute). The between-group differences (R minus E) were 5.2 mm Hg (P<0.0001) systolic, 1.3 mm Hg (P=0.12) diastolic, and 9.6 beats per minute (P<0.0001). In 57 R and 67 E patients with data available at all visits, these estimates were 5.5 mm Hg (P<0.0001) systolic, 1.8 mm Hg (P=0.07) diastolic and 9.8 beats per minute (P<0.0001). In R compared with E patients, 45 vs 37% (P=0.13) proceeded to the higher dose of randomised treatment and 33 vs 9% (P<0.0001) had α-methyldopa added. There were no between-group differences in symptoms except for ankle oedema in E patients (P=0.012). In conclusion, new compared with old drugs lowered systolic blood pressure more and therefore controlled hypertension better in native African black patients.

Cardiovascular risk factors in adult staff of Federal Medical Centre, Umuahia: a comparison with other Nigerian studies.

Changing lifestyle, driven by socio-economic trends, is changing the cardiovascular risk and disease profile in developing countries. We studied the cardiovascular risk factors of Federal Medical Centre, Umuahia hospital staff between 40 and 60 years. 297 members of hospital staff were consecutively recruited in October 2010. Data obtained include blood pressure, height and weight, body mass index (BMI), fasting blood sugar, serum cholesterol (total and differential) and serum triglyceride. The data were analysed using the SPSS package (version 10). The following prevalence levels were found: hypertension 37.5%, obesity 40.8%, diabetes mellitus 5.7%, hypercholesterolemia 18.1% and metabolic syndrome 24.7%. LDL, low HDL and elevated TG were elevated in 26.8%, 41.9% and 9.7% respectively. Only 2% had very low risk for cardiovascular disease.

Clinical and electrocardiographic evaluation of sickle-cell anaemia patients with pulmonary hypertension.

Pulmonary hypertension is an emerging complication of sickle cell anaemia with associated increased risk of mortality. In order to evaluate the clinical and electrocardiographic findings in adult sickle-cell patients with pulmonary hypertension, a cross sectional study was conducted on sixty two sickle cell anaemia patients and sixty two age and sex matched normal controls. Elevated pulmonary artery pressures (PAP), defined by PAP ≥ 30 mm Hg on echocardiography, was demonstrated in 41.9% of patients with sickle cell anaemia and in 3.2% of the controls; χ(2) = 26.571, P < 0.001. Right ventricular hypertrophy, increased P-wave duration, QTc interval, and QTc dispersion were significantly associated with pulmonary hypertension. Significant correlation was found between mean PAP and (1) Frequency of crisis (Spearman correlation = 0.320; P = 0.011), (2) body mass index (Pearson's correlation = -0.297; P = 0.019), and (3) QTc interval (Pearson's correlation 0.261; P = 0.040). Pulmonary hypertension in adult sickle anaemia patients is associated with electrocardiographic evidence of right ventricular hypertrophy, and correlates significantly with frequency of vaso-occlusive crisis, and QTc interval. The observations by this study tend to suggest that these parameters could be useful for early detection and prevention of pulmonary hypertension in patients with sickle cell anaemia.

Electrocardiographic findings in sickle cell cardiovascular autonomic neuropathy.

BACKGROUND: There is a lack of data on the relationship between cardiovascular autonomic neuropathy (CAN) and electrocardiographic parameters in sickle cell anaemia. AIMS AND OBJECTIVES: The purpose of the study was to compare the electrocardiographic findings in adult sickle cell anaemia patients with CAN with those of patients without this complication. METHODS: A cross sectional study was done using 62 consecutively recruited sickle cell anaemia patients who met the inclusion criteria for the study. Cardiovascular autonomic dysfunction was determined based on abnormal values in at least two of five non-invasive tests: Valsalva manoeuver, heart rate variation during deep breathing, heart rate response to standing, blood pressure response to sustained hand grip, and blood pressure response to standing. The subjects were subsequently evaluated with electrocardiography. RESULTS: Sickle cell anaemia patients with CAN had statistically significantly increased P-wave duration (p < 0.001), PR-interval (p < 0.05) and QTc dispersion (p < 0.05) compared with patients without CAN. Significantly increased frequencies of Q waves and first degree atrio-ventricular block were found in patients with CAN than in those without CAN (p = 0.026, p = 0.014, respectively). Significant correlations were noted between the severity of CAN [number of abnormal autonomic function tests (AFT)] and (1) P-wave duration (p = 0.008), (2) PR- interval (p = 0.013). Significant association was found between the number of abnormal AFT and (1) presence of Q-waves, and (2) degree of anaemia (haematocrit class). CONCLUSION: Electrocardiographic features consistent with atrio-ventricular and ventricular repolarization abnormalities are associated with CAN in sickle cell anaemia. Further studies are required to evaluate the prognostic implications of these findings in sickle cell patients with cardiovascular autonomic dysfunction.

Changes in lipids and lipoprotein indices in relation to the severity of hypertension in newly diagnosed hypertensive nigerians.

Hypertension and dyslipidaemia are important components of metabolic syndrome and both are known to complicate each other. Materials and Methods. A total of 149 subjects consisting of 107 hypertensive patients, grouped into 3 (of 37, 35, and 35 patients categorized based on the grade of hypertension as grade 1, grade 2, and grade 3, resp.) and 42 controls, were recruited for this study. Each subject had a recording of the bio- and anthropometric data comprising of the age, height, weight, body mass index (BMI), and abdominal circumference (AC). The blood pressure was also recorded. Fasting blood was collected and serum was used for the estimation of the lipids: total cholesterol (TC), high-density lipoprotein cholesterol (HDL-C), and triglyceride (TG), while low density lipoprotein cholesterol (LDL-C) and VLDL were estimated using Friedewald formula. Findings. Patients with hypertension had higher lipid and lipoprotein levels than the controls and the values became more significant with increasing severity of hypertension. The difference was statistically significant for TC, LDL-C, and VLDL-C (P < 0.05). Conclusion. This study showed that lipid and lipoprotein cholesterol abnormalities exist and even worsen with severity of hypertension. It is important that investigations in patients with hypertension should include a lipid profile.

Cardiac arrhythmias in recently diagnosed hypertensive patients at first presentation: an electrocardiographic-based study.

Various forms of cardiac arrhythmias have been documented in hypertensive subjects, and hypertension is an important risk factor for the development of atrial and ventricular arrhythmias and sudden death. Electrocardiography at rest easily documents significant arrhythmias in patients, and this study was carried out to determine the types and frequency of arrhythmias in hypertensive subjects at first presentation in the Hypertension Clinics of the University of Nigeria Teaching Hospital (UNTH) Enugu, Nigeria. The study was hospitalbased and retrospective in nature. The resting 12lead ECG reports of 346 consecutive hypertensive subjects seen at the Hypertension clinics of the UNTH Enugu over a 14 month period were retrieved from the case files and studied. Other information obtained from the case files included the age and gender of the subjects. The mean age of the subjects was 57.3 years. Ninety-five of the subjects had arrhythmias representing 27% of the study population, out of which fifty-five were males (57.9%) and forty were females (42.1%). However 26.9% of all the male subjects had arrhythmias while 28.2% of all the females had arrhythmias. Multiple ventricular ectopics, sinus tachycardia, sinus bradycardia and atrial fibrillation were the most prevalent arrhythmias. This study showed that a significant proportion of hypertensive subjects present initially with significant rhythm disturbances.

Atrial septal defects presenting initially in adulthood: patterns of clinical presentation in enugu, South-East Nigeria.

This paper aimed to evaluate the patterns of clinical presentation of adults with atrial septal defects (ASDs) who were diagnosed from transthoracic echocardiographic examination at the echocardiographic laboratory of the University of Nigeria Teaching Hospital Ituku-Ozalla, Enugu, Nigeria, from February 2002 to June 2010. 2251 new echocardiogram scans, with additional 373 repeat scans, were done within the period. 32 adults had ASDs (1.3%), made up of 9 males and 23 females. Secundum ASD constituted 75% while dyspnoea on exertion was the commonest symptom. Congestive cardiac failure was the clinical syndrome most commonly encountered, and most patients presented in the third decade. This paper demonstrated that ASDs are common congenital heart diseases in adult Nigerians, and that they are important causes of congestive heart failure. All adults with congestive heart failure must be referred for echocardiography for early identification of causes like ASDs, which are often forgotten, before the development of irreversible changes in the lungs.

Evaluation of blood pressure and indices of obesity in a typical rural community in eastern Nigeria.

AIM: With increasing urbanization of lifestyle, cardiovascular morbidity and mortality have been on the increase in Africans. Studies on cardiovascular risk factors in rural communities in South East Nigeria are scarce. This study focused on hypertension and obesity in adult Nigerians dwelling in a rural setting in Eastern Nigeria. MATERIALS AND METHODS: A total of 218 participants from the rural community were recruited into the study. A questionnaire was used to assess prior knowledge of their weight and blood pressure status as well as drug history for those found to have hypertension. Each participant's blood pressure was measured and any value ≥140/90 mmHg was regarded as high blood pressure (HBP). Their heights and weights were measured and their body mass indices (BMI) calculated using the standard formula of BMI = Weight in Kg/Height in m 2 ; BMI ≥30 Kg/m 2 was referred to as global obesity. Their waist circumferences (WC) were also measured and any value ≥102 cm for males and ≥88 cm for females was regarded as abdominal obesity. RESULTS: The general prevalence of HBP in the rural community was 44.5%. The prevalence of HBP increased as age increased and awareness about HBP was low (15.2%). Females were more aware than the males. The prevalence of HBP was higher in males (49.3%) compared with their female counterparts (42.3%), whereas the females had a higher prevalence of all forms of obesity (abdominal: 36.2%, global: 14.8%) compared with the males (abdominal: 14.5%, global: 10.1%). Higher BMI was associated with higher systolic and diastolic BP values. Hypertensive participants had higher BMI and WC than those who had normal BP. CONCLUSION: The prevalence of both hypertension and obesity seems to be increasing in rural communities in Nigeria and thus, the available prevalence documented in previous studies for rural communities may no longer represent the current trend. Awareness of the participants about these major cardiovascular risk factors is still very low. Higher BMI was associated with higher values of both systolic and diastolic BP.

P-wave dispersion: relationship to left ventricular function in sickle cell anaemia.

BACKGROUND: The prognostic implications of P-wave dispersion in patients with a variety of cardiac disease conditions are increasingly being recognised. The relationship between P-wave dispersion and left ventricular function in sickle cell anaemia is unknown. OBJECTIVE: This study was aimed at evaluating the relationship between P-wave dispersion and left ventricular function in adult Nigerian sickle cell anaemia patients. METHODS: Between February and August 2007, a total of 62 sickle cell anaemia patients (aged 18-44 years; mean 28.27 ± 5.58) enrolled in the study. These were drawn from patients attending the adult sickle cell clinic of the University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu. An equal number of age- and gender-matched normal subjects served as controls. All the participants were evaluated with electrocardiography and echocardiography. P-wave dispersion was defined as the difference between the maximum and minimum P-wave duration measured in a 12-lead electrocardiogram. RESULTS: P-wave duration and P-wave dispersion were significantly higher in patients than in controls. Significant correlation was demonstrated between P-wave dispersion and age in the patients (r = 0.387; p = 0.031). A comparison of subsets of sickle cell anaemia patients and controls with comparable haematocrit values (30-35%) showed significantly higher P-wave duration and P-wave dispersion in the patients than in the controls. The P-wave duration in patients and controls, respectively, was 111.10 ± 14.53 ms and 89.14 ± 16.45 ms (t = 3.141; p = 0.006). P-wave dispersion was 64.44 ± 15.86 ms in the patients and 36.43 ± 10.35 ms in the controls (t = 2.752; p = 0.013). Significant negative correlation was found between P-wave dispersion and left ventricular transmitral E/A ratio (r = -0.289; p = 0.023). CONCLUSION: These findings suggest that P-wave dispersion could be useful in the evaluation of sickle cell patients with left ventricular diastolic dysfunction. Further prospective studies are recommended to evaluate its prognostic implication on the long-term disease outcome in sickle cell disease patients.

Congestive heart failure in subjects with thyrotoxicosis in a black community.

INTRODUCTION: Thyroid hormone has profound effects on a number of metabolic processes in virtually all tissues but the cardiovascular manifestations are prominent usually creating a hyperdynamic circulatory state. Thyrotoxicosis is not a common cause of congestive heart failure among black communities. OBJECTIVES: To determine the hospital prevalence, clinical characteristics and echocardiographic findings in patients with thyrotoxicosis who present with congestive heart failure (CCF) in the eastern part of Nigeria. SUBJECTS AND METHODS: A total of 50 subjects aged 15 years and above who were diagnosed as thyrotoxic following clinical and thyroid function tests were consecutively recruited. Fifty age- and sex-matched controls with no clinical or biochemical evidence of thyrotoxicosis and no comorbidities were used as controls. Two-dimensional echocardiography was carried out on all the subjects. CCF was determined clinically and echocardiographically. RESULTS: Eight patients (5 females and 3 males) out of a total of 50 thyrotoxic patients presented with congestive heart failure. CONCLUSION: The study revealed that congestive heart failure can occur in thyrotoxicosis in spite of the associated hyperdynamic condition. The underlying mechanism may include direct damage by autoimmune myocarditis, congestive circulation secondary to excess sodium, and fluid retention.

Arterial blood pressure in adult Nigerians with sickle cell anemia.

AIM AND OBJECTIVE: This study was aimed at comparing the arterial blood pressures in steady state adult sickle cell patients with those of age- and sex-matched healthy controls. METHODS: A descriptive cross-sectional study of 62 sickle cell anemia patients and 62 age- and sex-matched healthy controls was carried out in the adult outpatient sickle cell clinics and the cardiac center of the University of Nigeria Teaching Hospital (UNTH), Enugu, Nigeria. Brachial blood pressures were measured in the right arm in all subjects. RESULTS: Significant increase in pulse rate was found in the study subjects (87.68 ± 8.91 bpm) compared with the controls (72.13 ± 6.79 bpm) (p<0.05). The mean systolic blood pressure was comparable in the two groups. However, the patients had significantly lower diastolic blood pressure, lower mean arterial blood pressure, as well as a higher pulse pressure than the control subjects. Significant correlations were found between blood pressure indices and hematocrit, body mass index, frequency of crisis, and body surface area. CONCLUSION: Relatively lower arterial blood pressure is a significant finding in patients with sickle cell anemia. Hematocrit, frequency of crisis, body mass index, and body surface area are significant determinants of blood pressure indices in sickle cell anemia.

Electocardiographic findings in adult Nigerians with sickle cell anaemia.

BACKGROUND: Cardiovascular system abnormalities are common causes of morbidity and mortality in sickle cell anaemia. OBJECTIVES: The study aims at determining the pattern of electrocardiographic changes in adult Nigerian sickle cell anaemia patients. METHODS: A descriptive cross sectional study was done on sixty sickle cell anaemia patients seen at the adult sickle cell clinic of University of Nigeria Teaching Hospital (UNTH) Enugu, and sixty age and sex matched normal controls. All the subjects had clinical evaluation as well as electrocardiographic examination. RESULTS: The mean heart rate, P-wave duration, P-wave dispersion, PR interval, QRS duration, QRS dispersion, QTc interval and QTc dispersion were significantly higher in the patients than in the control group. Electrocardiographic abnormalities identified by this study were: left ventricular hypertrophy (75%; 1.7%), left atrial enlargement (40%; 0%), biventricular hypertrophy (11%; 0), ST-segment elevation (10%; 0%) and increased P-wave and QTc dispersions. ST segment elevation was found more in patients with moderate and severe anaemia (P= 0.02, Spearman correlation r= 0.342; P= 0.007), CONCLUSION: Sickle cell anaemia is associated with significant electrocardiographic abnormalities. Further prospective studies are recommended to evaluate the prognostic significance of the electrocardiographic intervals dispersion on the long term disease outcome in sickle cell anaemia.

Ventricular septal defects at the University of Nigeria Teaching Hospital, Enugu: a review of echocardiogram records.

This research aimed to evaluate the prevalence of ventricular septal defect (VSD) and to determine the age and gender distribution of this defect and its common associated congenital cardiac anomalies among patients referred for echocardiographic examination at the echocardiography laboratory of the University of Nigeria Teaching Hospital, Enugu, Nigeria. Echocardiogram reports from the 10-year period February 1991-September 2001 were analysed. There were 2486 echocardiogram scans with an additional 334 repeat scans; 593 subjects had congenital anomalies, of which 165 (28%; 98 males and 67 females) had VSDs. The highest prevalence of VSDs (118; 70%) was in children age 0-9 years although VSDs were found in adults aged up to 50 years and above. Persistent ductus arteriosus and atrial septal defect were the commonest associated congenital anomalies. The study has shown that VSD is a relatively common congenital heart disease and that many patients present with VSD in adult life. There is a need for thorough scrutiny to be made for other cardiac anomalies in all patients with VSD, as well as for corrective surgery to be made affordable to those affected by this defect and other associated anomalies.

One year echocardiographic study of rheumatic heart disease at Enugu, Nigeria.

BACKGROUND: Rheumatic heart disease is one of the commonest causes of heart disease in Nigeria. Previous studies on rheumatic heart disease in Nigeria were either clinical or retrospective echocardiographic studies. This study was aimed at determining the burden of chronic rheumatic mitral valve disease, pattern of valvular involvement, severity and associated valvular lesions, using echocardiography. PATIENTS AND METHODS: Between May, 2004 and April 2005, patients with symptomatic rheumatic heart disease, seen in the cardiac clinics of University of Nigeria Teaching Hospital, Enugu, were recruited for the study. The patients were investigated non-invasively with M-mode, Two-dimensional, Pulsed wave, Continuous wave and colour flow Doppler echocardiographic techniques in order to assess the mitral and other intracardiac valves for evidence of rheumatic heart disease. RESULTS: There were a total of 55 patients, 39 (70.9%) females and 16 (29.1%) males with a mean age of 29.34 +/- 11.57 years. Mitral valve disease was found in 54 (98.2%) and isolated aortic valve disease in 1 (1.8%) of cases. Mitral regurgitation occurred in 64.8%, mixed mitral valve disease in 25.9% and pure mitral stenosis in 9.3%. Mitral valve disease was associated with aortic valve disease in 33.3%, with tricuspid valve disease in 24.1% and with functional pulmonary incompetence in 9.3% of cases. CONCLUSION: Rheumatic heart disease in Nigeria is essentially a disease of the mitral valve as seen elsewhere in the world. Echocardiography should be done routinely for patients with rheumatic heart disease to facilitate accurate diagnosis and definitive treatment.

Facioscapulohumeral muscular dystrophy (landouzy-dejerine type) in a Nigerian female: a case report.

BACKGROUND: Muscular dystrophy is not an uncommon entity in Nigerian medical clinics. The facioscapulohumeral type represents a rare variety of the disorder with its own distinctive characteristics but is not expected to have significant cardiac manifestations. METHOD: The case report of a 17-year-old Nigerian female with facioscapulohumeral muscular dystrophy and significant cardiac dysfunction is presented and the relevant literature is reviewed. RESULTS: A 17-year-old Nigerian female presented with 18 month history of shortness of breath on mild to moderate exertion, generalized weakness, weight loss and abnormal gait. Leg swelling developed a few weeks prior to presentation. Intrauterine life and early childhood were uneventful while her family history was unremarkable. She had typical features of facioscapulohumeral muscular dystrophy but in addition demonstrated evidence of significant cardiac impairment, which is uncommon and not typically expected in this disorder. CONCLUSION: It is important for clinicians to comprehensively evaluate every patient presenting with a hitherto "clear "diagnosis in order to unmask unexpected associated clinical details.