A case of a 42-year-old patient with anomalous origin of the left main coronary artery from the pulmonary artery who delivered three times with no complications: presentation, diagnosis, and review.

An anomalous origin of the left coronary artery (LCA) from the pulmonary artery or Bland-White-Garland (BWG) syndrome is a rare congenital cardiac anomaly, which is unusual to survive to adulthood if left uncorrected. The authors report an unusual case of a multiparity patient who delivered several times without any complications presenting at the age of 42 years with recurrent dyspnea on exertion in which echocardiographic findings of diastolic flow near the origin of the pulmonary valve were suggestive of anomalous origin of the LCA. A CT angiography confirmed that the left main coronary artery arises from the undersurface of the pulmonary flow close :o its origin and gives rise to a left anterior descending (LAD) and left circumflex arteries.

Diabetes supersedes dobutamine stress echocardiography in predicting cardiac events in female patients.

BACKGROUND: The many available choices for testing for coronary artery disease (CAD) brought about several questions regarding suitability of certain tests for different groups of patients and the prognostic value of obtained results in predicting events and mortality. The aim of this study is to describe the prognostic value of dobutamine stress echocardiography (DSE) results in predicting cardiac events and mortality in > or = 60-year-old females. METHODS: 49 women (> or = 60 years old) who were referred for DSE were included in the study. Data including CAD risk factors, and results of tests and a follow-up of events (MI, unstable angina, progression of CHF) and death. RESULTS: Eleven patients were considered to have a positive DSE result. There was no difference between DSE (+) and DSE (-) patients in cardiac events and cardiac death. However when interventions were included to events, analysis showed DSE (+) to have more overall events. Non-cardiac deaths and "all deaths" were 11 and 8 times more common among DSE (+) patients compared with DSE (-) patients p < 0.01. Multivariable logistic regression showed that diabetics and DSE (+) patients were 32 (p = 0.01) and 23 (p = 0.02) times more likely to have an event compared with non-diabetics and DSE (-) patients, respectively. CONCLUSION: DSE is a safe procedure to be used in > or = 60-year-old female patients and can provide informative prognostic information regarding all-cause deaths and cardiac events (including interventions) over a 4-year period. In addition we find that diabetes is a strong predictor of events regardless of DSE result.

Screening for inherited thrombophilia might be warranted among Eastern Mediterranean sickle-beta-0 thalassemia patients.

BACKGROUND: The role of genetic thrombophilia in the development of both micro and macro vascular complications in patients with hemoglobinopathies (Sickle cell disease and thalassemia) have been investigated with some studies negating its role while others suggesting it. Lebanon is known to harbor sickle cell disease, thalassemia and sickle beta-thalassemia hemoglobinopathy patients along with a documented high prevalence of genetic thrombophilia mutations. METHODS: Twelve sickle beta-0-thalassemia patients with no pervious history of thrombotic events were selected. These patients underwent a physical examination with history, echo Doppler, along with blood withdrawal for complete blood count and PCR analysis of a sample of DNA for Factor V Leiden G1691A, Factor II G20210A, and MTHFR C677T. Results were compared to a historical control of 50 Lebanese controls and 50 LebaneseThalassemia Intermedia (TI) patients. RESULTS: The results showed that 42%, 59%, and 8% of patients carried heterozygous Factor V Leiden, abnormal (homozygous & heterozygous) MTHFR, and heterozygous Factor II mutations respectively. The sickle-thalassemia patients were 5.24 and 4.39 times more likely to have Factor V Leiden as compared to the normal controls and TI patients respectively (p < 0.05). DISCUSSION: The increased prevalence of more than one prothrombotic genetic mutation among the group indicates a probable clustering phenomenon, unknown to us to which the high consanguinity rate (77%) may have contributed. The role of the specific MTHFR and Factor V Leiden double heterozygous combination in incidence, recurrence, and guidance of duration of therapy in VTE is not well defined in the literature despite the recognized higher risk of thrombosis among this patient population. Our findings suggest that genetic thrombophilia workup is necessary in patients with sickle-beta zero thalassemia presenting with thrombotic events and studies that include a larger number of patients are necessary in order to define specific guidelines.

Genetic thrombophilia in patients with VTE in eastern Mediterranean located tertiary care center; is it time to change the algorithm for thrombophilia work up decision making?

BACKGROUND: Genetic thrombophilia work up performance is subject to debate because of the utility of the information it provides regarding prognosis of recurrence of venous thromboembolism (VTE) and guidance of duration of therapy with anticoagulation. Certain algorithms have been locally developed to guide decision making process to increase the yield of thrombophilia work up. These algorithms are not based on locally derived data. Data from the Eastern Mediterranean area (Lebanon) have shown high prevalence of thrombophilia mutations. Therefore the aim of this study was to describe the experience of a tertiary care center in thrombophilia work up among patients diagnosed with VTE. METHODS: A retrospective chart analysis of the cases diagnosed with pulmonary embolism or deep venous thrombosis with radiological confirmation. RESULTS: A total of 133 patients' charts were reviewed, 27 patients had thrombophilia work up performed, 56% had heterozygous MTHFR mutation, 44% were heterozygous for the Factor V Leiden mutation and 3.2% were found to have Factor II heterozygous mutations, and a total of 33.3% of patients had more than one genetic mutation. The common causes of provoked VTE were 59.53% malignancy, 22.64% post surgery, 12.98% bed ridden patients, and 4.85% oral contraceptive pills. Only 14% of patients with a malignancy who presented with VTE where receiving prophylactic heparin. DISCUSSION: Doubly heterozygous prothrombotic genetic mutations are commonly present among Eastern Mediterranean patients with VTE. A prospective study to determine the predictive (negative and positive) power of the currently followed algorithm for genetic thrombophilia work up remains of significant importance. Stressing the favorable role of VTE prophylaxis among patients suffering from malignancies remains a target for raising awareness among oncologists.

Peripartum cardiomyopathy: characteristics and outcome in a tertiary care hospital.

PURPOSE OF INVESTIGATION: Peripartum cardiomyopathy (PPCM) has been traditionally regarded as a condition with a poor prognosis. We studied the acute and long-term outcomes in patients with PPCM in a tertiary care hospital. METHODS: Patients with PPCM admitted to our hospital from June 1990 to February 2002 who had documented left ventricular (LV) dysfunction by echocardiographic criteria were retrospectively reviewed. RESULTS: Out of ten patients who fulfilled the criteria for the diagnosis, six (60%) had severe, and four (40%) had moderate LV dysfunction at echocardiography. None had evidence of other chronic disease. Treatment consisted of fluid restriction, diuresis, and afterload reducers in all, and intravenous inotropes in three (30%) of the patients. No patient died while in hospital or during follow-up. All patients showed improvement in their clinical condition and LV function on follow-up which normalized in seven (70%). CONCLUSION: PPCM might carry a relatively good prognosis in patients with absence of associated disease conditions.

Influence of angiotensin converting enzyme inhibitors on stable myocardial ischemia in menopausal cardiac patients.

OBJECTIVE: The aim of the study was to investigate the influence of angiotensin converting enzyme inhibitor (Captopril) on stable demand ischemia in menopausal cardiac female patients. METHODS: In a prospective non-randomized evaluation of the effect of angiotensin converting enzymes inhibitors (ACE-I) (Captopril) on demand myocardial ischemia, 16 normotensive menopause female patients, mean age of 52 years with stable angina and known coronary artery disease (CAD) but normal left ventricular function, underwent a treadmill exercise test (Bruce Protocol), at baseline (T1) and one week following (50-75 mg) a daily dose of Captopril (CAPT2). Onset of symptoms, duration of exercise, magnitude of peak ST depression and homodynamic parameters were monitored. RESULTS: Captopril significantly increased the duration of exercise from (467 +/- 169 to 536 +/- 145 seconds, (p value < 0.02), but with increased peak of ST segment depression (from -1.4 +/- 0.6 mm to -1.93 +/- 1.2 mm, p value < 0.15). The double product remained unchanged (251 x 103 +/- 55 in T1 and 248 x 103 +/- 55 in CAPT2; the p value was < 0.8. All adverse effects on the treadmill were noted. CONCLUSION: Although captopril tends to significantly effect prolongation of exercise time, there is no amelioration of the markers of ischemia.

C-reactive protein is a marker for a complex culprit lesion anatomy in unstable angina.

BACKGROUND: The putative theory is that the clinical syndrome of unstable angina is caused by rupture of the atherosclerotic plaque with superimposed thrombus formation. It is characterized by angiographically complex coronary lesions in the majority of patients. HYPOTHESIS: This study aimed at assessing the correlation between C-reactive protein (CRP) and the complexity of culprit coronary lesions in unstable angina. METHODS: We identified culprit lesion complexity in 96 patients with unstable angina and normal creatine kinase (CK) and CK-MB mass. Serum concentrations of CRP (N < 5.0 mg/l) and cardiac troponin T (cTnT; N < 0.1 ng/ml) were measured on admission. RESULTS: There was a trend toward a higher grade of anatomical complexity of the culprit lesion in patients with elevated CRP (p = 0.007) and cTnT levels (p = 0.027). Patients who had intermediate- or high-grade lesion severity had a higher level of CRP (8.5 +/- 5.7 mg/l) and cTnT (0.118 +/- 0.205 ng/ml) on admission than those who had normal or low-grade lesions (5.7 +/- 4.0 mg/l, 0.017 +/- 0.021 ng/ml, respectively); Mann-Whitney U, p = 0.002 and p < 0.001, respectively. Furthermore, the likelihood of having intermediate- or high-grade complexity of the culprit lesion was higher when CRP levels were elevated in all patients (p = 0.007, odds ratio [OR] = 4.286; 95% confidence interval [CI] 1.492-12.310) and in those with normal cTnT levels (p = 0.025, OR = 3.876; 95% CI 1.185-12.678). Also, higher CRP levels strongly correlated with the need for revascularization interventions (p < 0.0005). CONCLUSION: Elevated CRP level on admission is a marker for anatomic complexity of culprit lesions and need for revascularization interventions in unstable angina.

Unusual manifestation of an ampullary tumor presenting with severe upper gastrointestinal bleeding.

Ampullary tumors can occasionally ulcerate and present as frank gastrointestinal bleeding. The most common clinical presentation is jaundice like in other tumors of the biliary tree. We report on a 68-year-old man who presented with severe upper gastrointestinal hemorrhage secondary to an asymptomatic mass of the ampulla of Vater. An endoscopic biopsy specimen revealed a villous adenoma with moderate dysplasia. A curative resection was performed, and pathological work-up revealed the presence of an infiltrating, moderately differentiated ampullary adenocarcinoma.

Is there a safe anticoagulation protocol for pregnant women with prosthetic valves?

We studied the outcome of 41 pregnancies in an attempt to identify an appropriate and safe anticoagulant regimen for pregnant women with cardiac valve prosthesis. The cumulative number of events: (neonatal death, premature deliveries, intrauterine fetal death and spontaneous abortion) was 66.6%, 20%, 12.5% and 20% in the Coumadin, Fraxiparine, Heparin and porcine valve groups, respectively. We conclude that in pregnant women with mechanical heart valves, low molecular weight heparin therapy is a superior strategy compared to warfarin.