Clinical case of Cor triatriatum sinister, a dilemma of anticoagulation: A case report and literature review.

Cor triatriatum is a rare congenital heart abnormality in which a membrane separates the left atrium (LA; sinister) or the right atrium (dexter) into two compartments. It is also a long-forgotten cause of atrial fibrillation (AF) and substantially higher rates of blood stagnation, particularly proximal to the additional septum in the LA. In this case report, we faced a CHA2DS2-VASc score of 1 in patients with non-valvular AF due to Cor triatriatum sinister (CTS). The decision to start anticoagulants in this particular case was controversial, so we reviewed the literature to assess and address it. We present our case and discuss the indication of anticoagulants in this unique clinical scenario, accompanied by a literature review. Facing this dilemma of starting anticoagulants in special cases of CTS and AF should be individualized and need more investigation. However, till this moment, based on similar reports, it seems to be rational to consider CTS Per se as an additional risk stratification marker beyond the CHA2DS2-VASc score start anticoagulant until the surgical resection. Considering CTS as the sole indication of anticoagulant in patients with normal sinus rhythm is a complex matter that needs further investigation.

Reduced myocardial reserve in cirrhotic patients: an evaluation by dobutamine stress speckle tracking and tissue Doppler imaging (TDI) echocardiography.

Introduction: Despite the normal systolic function at rest, cirrhotic patients often suffer from volume overload and symptoms of heart failure as they face stressful situations. This study investigated the myocardial reserve in cirrhotic patients at resting condition and peak stress by dobutamine speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI). Methods: Twenty cirrhotic patients and 10 normal individuals aged 30-50 were selected randomly. For all of the participants, complete echocardiographic study of 2D, STE and TDI was done at rest and peak stress status with dobutamine. The following parameters were assessed: ejection fraction (EF), global longitudinal LV strain (GLS), strain rate in the septal basal segment and lateral wall and E' in the septal basal segment by color-coded method. Results: At baseline, EF was higher than 55% in both groups. GLS was higher (-22.6±2.4%) in the case group than the control group (-19.2±1.9%) at resting condition. After stress, it showed a greater increase (-22.5±1.7%) in the controls compared to cirrhotic patients (-22.6±3.3%; mean difference = 2.6 ± 2.03, P = 0.02). In cirrhotic patients, the average strain rate in the basal septal segment decreased after stress (-1.2 ± 0.3/s to-1.1 ± 0.3/s), but it increased in the control group (-1.1 ± 0.2/s to -1.8 ± 0.2/s). Conclusion: Despite the presence of normal resting systolic function in cirrhotic patients, there was insufficient increase or even a decrease in myocardial function with stress; this may indicate the absence of sufficient myocardial reserve in cirrhotic patients. These findings would help to explain the reason for occurrence of heart failure or hemodynamic changes in cirrhotic patients.

Myocardial contractile dispersion: A new marker for the severity of cirrhosis?

Introduction: Cirrhotic cardiomyopathy (CCM) develops in about half of all cirrhotic patients, affecting the long-term morbidity and mortality. Although some studies have shown an increased QT-interval in cirrhotic patients, no evidences of myocardial contractile and QT dispersion (QTd) changes are available. This study aimed to compare myocardial contractile dispersion (MCd), using tissue Doppler imaging (TDI), as well as QTd between cirrhotic patients and healthy individuals, investigating their associations with cirrhosis severity. Methods: This prospective cross-sectional study was conducted on patients with confirmed liver cirrhosis and healthy individuals. Participants with structural heart disease, heart ventricular pacing, electrolyte abnormalities, using drugs affecting QT interval were excluded. All individuals underwent 2D echocardiography, and TDI by vivid E9 echo machine. MCd and QTd were considered as main outcomes. Chi-square, independent-sample t test, and Pearson correlation test, were used for statistical analyses by SPPS version 17.0. P value <0:05 was considered statistically significant. Results: Sixty participants (40 male/20 female) with a mean age of 40.1 ± 7.1 years in two groups of cirrhotic patients (n=30) and healthy individuals (n=30) were studied. Both groups were statistically similar in terms of age (P = 0.31) and gender (P = 0.39). MCd and QTd of cirrhotic patients were significantly higher than healthy individuals (MCd: 41.0 ± 26.8 versus 27.6±18.1; P = 0.028; and QTd: 37.0 ± 22.1 versus 25.3 ± 8.9; P = 0.010). Cirrhotic patients with MELD score <15 had a lower MCd in comparison to score ≥15 (29.2 ± 13.8 versus 50.0 ± 31.1, P = 0.034). Conclusion: Cirrhosis was associated with increased MCd, assessed by TDI. Also, MCd and QTd were associated with a higher MELD score. According to the results, it seems that MCd and QTd might be useful predictor of ventricular arrhythmia and negative prognostic factor in cirrhotic patients.

Almond oil for patients with hyperlipidemia: A randomized open-label controlled clinical trial.

BACKGROUND: Cardiovascular diseases currently account for nearly half of non-communicable diseases. It was shown that enjoying a handful of nuts every day can significantly reduce the risk of developing heart diseases as they contain a variety of nutrients and other bioactive substances contributing to lowering the risk of heart diseases and controlling the cholesterol. The aim of this study was to determine the effect of almond oil on the lipid profile of patients with hyperlipidemia. METHODS: Ninety-seven patients were divided into the intervention (n = 49) and control (n = 48) groups. The intervention group received 10 ml of almond oil two times daily for 30 days. There was no intervention for the control group. The serum lipoproteins were measured before and after the study. RESULTS: The total cholesterol and LDL levels decreased significantly in the intervention group (treatment difference = -16.12 ± 26.16, P = 0.009; treatment difference = -20.88 ± 18.4, p < 0.001 respectively). But regular almond oil consumption did not significantly affect the triglyceride and HDL in this sample of hyperlipidemic patients. CONCLUSION: Consumption of almond could reduce the total cholesterol and LDL in dyslipidemic patients.

Assessment of Subclinical Myocardial Changes in Non-Alcoholic Fatty Liver Disease: A Case-Control Study Using Speckle Tracking Echocardiography.

BACKGROUND: Considering the association between cardiac abnormalities and non-alcoholic fatty liver disease (NAFLD), the present study aimed to evaluate the relationship between biopsy-proven NAFLD and functional echocardiographic parameters, including left ventricular (LV) global longitudinal strain (GLS) in asymptomatic individuals. METHODS: Thirty asymptomatic patients with liver biopsy-proven NAFLD and the same number with no evidence of fatty liver in ultrasonography were enrolled in the study as cases and controls, respectively. The measured echocardiographic parameters included LV ejection fraction (LVEF), LV end-systolic and end-diastolic dimensions (ESD, EDD), LV end-systolic and end-diastolic volumes (ESV, EDV), E/e' ratio (early-diastolic mitral inflow velocity/early-diastolic myocardial velocity), E/A ratio (early-diastolic mitral inflow velocity/late-diastolic mitral inflow velocity), and GLS. Data were analyzed using the SPSS statistical software (version 18.0) by performing the independent t test, Chi-square, and non-parametric Mann-Whitney U tests. P values <0.05 were considered statistically significant. RESULTS: A significant difference in ESD (32.1±1.4 mm vs. 34±1.8 mm), EDD (41.9±1.7 mm vs. 45.2±3.1 mm), and E/e' ratio (8.4±0.8 vs. 7.4±1.2) was detected among individuals with NAFLD compared with those without NAFLD (P<0.001 for the first two parameters and P=0.002 for the last one). GLS was also significantly lower in NAFLD patients than in controls, but within normal levels (19.3%±2.0 vs. 21.2%±1.4, P<0.001). CONCLUSION: The findings support the presence of subclinical cardiovascular structural and functional changes in patients affected by NAFLD. It also indicates that the use of GLS is more sensitive than LVEF for the detection of LV systolic dysfunction in NAFLD patients.

The Correlation between Echocardiographic Findings and QT Interval in Cirrhotic Patients.

BACKGROUND: Although many electrocardiographic abnormalities have been reported previously, prolonged QTC interval represented as the most important ECG finding in patients with liver cirrhosis. Echocardiography can detect structural cardiac abnormalities in cirrhotic patients. OBJECTIVES: The present study aimed to determine the correlation between QTC prolongation and echocardiographic findings in end stage liver cirrhosis. METHODS: The present study was conducted on 100 patients selected through convenient sampling. We recruited 80 cirrhotic patients with CHILD score > B or MELD score > 15 from the transplantation ward of Nemazee educational hospital. A complete echocardiographic study, including chamber quantification, a complete flow Doppler, and tissue Doppler analysis, was performed for each patient using a GE vivid 3 system equipped with Tissue Doppler Imaging (TDI). Then, twelve-lead ECG was carried out and QTc interval was calculated in all patients. The data were analyzed using the SPSS statistical software (v. 13) and Pearson's correlation coefficient. P value < 0.05 was considered statistically significant. RESULTS: The patients' age ranged from 20 to 60 years old and 62.5% of them were male. According to the results, the only parameter which was significantly associated with prolonged QTc interval was Left Ventricular End Diastolic Dimension (LVEDD). Additionally, a linear direct relationship was found between corrected QT interval and LVEDD (r = 0.41, P < 0.001). CONCLUSIONS: The current study showed a positive correlation between QTC prolongation as an electerocardiographic finding and LVEDD in echocardiography of the cirrhotic patients. This may indicate a direct relationship between the electrophysiological problems and the severity of volume overload in cirrhotic patients.

Corrected QT interval and QT dispersion in cirrhotic patients before and after liver transplantation.

BACKGROUND: Liver cirrhosis is associated with different types of electrophysiological changes, including QT prolongation, which may adversely affect long-term prognosis of these patients. The aim of this study is to evaluate the effect of orthotopic liver transplantation (LT) on corrected QT (QTc) interval and QT dispersion (QTd) in cirrhotic patients of various etiologies. METHODS: We enrolled 249 patients with end-stage liver disease between 2004 and 2009 at Shiraz Transplant Research Center, Shiraz, Iran. The QTc interval and QTd were measured by 12 lead ECGs for baseline and at 3 months after LT. Mean QTc interval and mean QTd were calculated. A QTc interval above 440 ms was considered abnormal. RESULTS: Within 3 months following surgery, 6 patients died. There were 105 patients (43.2%) with prolonged QTc before transplantation; in 91 (86.6%) patients, the mean QTc normalized after transplantation (baseline: 490.9 ± 45.74 ms; post-transplantation: 385 ± 48.74 ms; P < 0.0001). Fourteen patients (13.3%) had evidence of some shortening of the QTc interval although the QTc remained above the upper limit of normal. Prolongation of the QTc interval in cirrhotic patients was independent of the etiology of cirrhosis. A normal QTc was seen in 138 patients (56.7%) before transplantation, of which 4 (2.9%) developed prolonged QTc after transplantation. The mean QTd decreased significantly after transplantation (baseline: 30 ± 20 ms; post-transplantation: 30 ± 10 ms; P < 0.0001). CONCLUSION: Many cirrhotic patients have prolonged QTc intervals before LT regardless of disease etiology. In the majority of patients this value returns to normal after LT, suggesting that liver cirrhosis has independent unfavorable, but reversible electrophysiological effects.

Liver cirrhosis prevents atrial fibrillation: A reality or just an illusion?

OBJECTIVES: In cirrhotic patients, despite presence of significant metabolic abnormalities and enlarged left atria, we have seldom observed atrial fibrillation (AF) rhythm. The aim of this study was to evaluate the prevalence of AF among cirrhotic patients and comparing it with that reported in the general population. MATERIALS AND METHODS: Medical documents of 1302 adult cirrhotic patients, booked in the waiting list of liver transplantation, were evaluated retrospectively. Electrocardiograms were reviewed for the presence of AF rhythm, and echocardiographies were reviewed to obtain left atrial (LA) size. RESULT: Only two patients (0.15%) were detected to have AF rhythm, which was significantly lower than that reported in our general population, even after age and sex adjustment. Six hundred and sixty-eight patients (51%) had enlarged left atria (LA diameter more than 39mm); both AF patients belonged to this group. CONCLUSION: The result of this study suggests the protective effect of liver cirrhosis on development of atrial fibrillation. Further studies are needed to verify the possible mechanisms.

Right ventricular thrombus: a rare complication of ovarian hyperstimulation syndrome.

A 22 years old lady was admitted because of progressive dyspnea, severe abdominal protrusion and lower extremity edema. She had undergone ovarian hyperstimulation for primary infertility by Clomiphen and Human chorionic gonadotropin for 3 months. Abdominopelvic ultrasonography revealed bilateral enlarged multi cystic ovaries and massive ascites. Transesophageal echocardiography revealed a large thrombus in right ventrice apex. Spiral chest CT scan showed normal pulmonary vasculature with no evidence of pulmonary thromboembolism. Heparin was started and repeat echocardiographic study showed gradual disappearance of right ventricular thrombus. Human chorionic gonadotropin is the most important substance which leads to capillary leakage and fluid accumulation in third space. Fluid shift and hypovolemia may cause hypotension, hemoconcentration and formation of vascular thrombus.

Tacrolimus related hypertrophic cardiomyopathy in liver transplant recipients.

BACKGROUND: Recently there are a number of reports on the cardiotoxicity of tacrolimus in post-transplant patients. There is no protocol for cardiovascular evaluation in these patients. This study was performed to evaluate the cardiotoxicity of tacrolimus in liver transplant recipients. METHODS: We evaluated 63 post-liver transplant patients who received tacrolimus. They were evaluated for cardiovascular complications by physical examination, electrocardiographic and echocardiographic examinations within three and six months following liver transplantation. Serum tacrolimus levels were checked by ELISA. For comparison, we selected 50 post-liver transplant patients who received no tacrolimus and evaluated them for cardiovascular function identically. RESULTS: Among 63 patients, 42 were male (66.7%) and 21 were female (33.3%); 70% of the patients were adults, and 19 (30%) were within the pediatric age group. The cardiovascular examinations, electrocardiogram and echocardiography of all patients three months post-transplantation were normal except for two children who developed tacrolimus related cardiac complications. Both had high serum tacrolimus levels. No adults developed cardiovascular complications. In the control group, the results of the cardiovascular evaluations were normal in all cases. CONCLUSION: The cardiovascular toxicity of tacrolimus, such as hypertrophic cardiomyopathy, may be observed in pediatric patients. Therefore, we recommend routine regular cardiovascular evaluation of children after liver transplantation.

Pulmonary complications in cirrhotic candidates for liver transplantation.

BACKGROUND AND AIMS: The determination of the prevalence of cardiopulmonary complications at a liver transplant center in Iran. METHODS: Ninety-nine patients (61 male and 38 female) with a mean age of 36.5 (15-66) years with proven cirrhosis were enrolled in this study. Patients with primary cardiac disease, current smokers, those with sepsis, hepatocellular carcinoma, recently ruptured esophageal varices and chronic pulmonary or renal diseases were excluded from the study. Sixty-nine patients had ascites. Forty-four patients had grade C Child-Pugh classification. All patients were evaluated for respiratory function by chest X-ray (CXR), room air arterial blood gas, simultaneous pulse oximetry, cardiac echocardiography and spirometry. RESULTS: Sixty-one patients (66.1%) had a widened alveolar-arterial O2 difference ( > 20 mmHg); 14 (14.1%) had hypoxemia; 6 (6.1%) had mean pulmonary arterial pressure (MPAP) = 25-40 mmHg; 12 (12.1%) had tricuspid regurgitation; pleural effusion and lung restriction were detected in 4 (4%) and 50 (50.5%), respectively. P(A-a)O2 was negatively associated with pulmonary hypertension (P < 0.03) and tricuspid regurgitation (P < 0.005). Portal hypertension and portal vein thrombosis were detected in 91 and 8 patients, respectively. CONCLUSIONS: A widened alveolar-arterial oxygen difference was common in our patients, but hypoxemia occurred in 14% of patients. Portopulmonary hypertension was preponderant in those patients of male gender.

Patent foramen ovale in patients with tension headache: is it as common as in migraineurs? An age- and sex-matched comparative study.

The association of patent foramen ovale (PFO) and atrial septal aneurysm (ASA) with migraine headache attack (MHA) has been clearly shown. The same findings have been recently demonstrated also in cluster headache. Although tension-type headaches (TTH) are the most common kind of headache, their association with these atrial septal abnormalities has never been studied before. The study was conducted to clarify whether there was a significant association between the presence of such atrial septal abnormalities and tension headache, when compared with migraineurs. One hundred consecutive patients with migraine and 100 age- and sex-matched subjects with TTH and 50 healthy volunteers with no headache were enrolled in the study and underwent a complete transesophageal echocardiographic study with contrast injections at rest and with the Valsalva maneuver. There was no significant difference between the age and the sex of the participants of the three groups. The overall prevalence of PFO was 23% in patients with TTH and that of large PFOs was only 11%. The 23% prevalence of PFO in patients with TTH was not statistically different from 16% found in our normal control group. Furthermore, we found a significantly higher prevalence of PFO in migraineurs (50%) when compared with patients with tension headache (p < 0.001). This was also true for the collective presence of large PFOs and ASAs (35%) (p < 0.001). Although atrial septal anomalies have an association with MHA, they do not have a significant association with TTH.

Aortic valve replacement in a patient with liver cirrhosis and coagulopathy.

Open heart surgery in patients with end-stage liver disease is accompanied by various complications. Coagulopathy resulting from liver failure tends to cause uncontrollable hemorrhage. Severe aortic insufficiency has moderate to severe risk for liver transplantation. It can lead to heart failure, liver congestion, and finally rejection of the transplanted liver. Aortic valve replacement in patients diagnosed as having cirrhosis has a significant risk of mortality because of the above-mentioned complications. We present a patient with liver cirrhosis and severe aortic insufficiency who had thrombocytopenia and severe coagulopathy. Aortic valve replacement was performed successfully using cardiopulmonary bypass before the liver transplantation. Hemostasis management was done effectively perioperation. The postoperative course was uneventful, and the patient was discharged after 10 days. Liver transplantation was performed successfully 2 months later.

Left ventricular to right atrial communication (Gerbode-type defect) after mitral valve replacement.

A 51-year-old woman with severe rheumatic mitral stenosis underwent mitral valve replacement. One week later, she was admitted with abdominal pain and lower extremities edema. On physical examination, her blood pressure was 120/60 mm Hg and holosystolic murmur (3/6) was heard at the left sternal border. Transesophageal echocardiography was done and revealed a shunt flow from the left ventricle to the right atrium in the 4-chamber and bicaval views. With careful examination of the interventricular septum, a small defect was seen in the membranous interventricular septum, which connects the left ventricle to the right atrium. The patient underwent surgical operation with diagnosis of iatrogenic left ventricular to right atrial communication (Gerbode-type defect). Intraoperative finding confirmed the preoperative diagnosis.

Acquired accessory left atrium.

The patient was a 61-year-old woman brought to our hospital due to dyspnea and lower extremity swelling in shock state three weeks after mitral valve replacement due to rheumatic mitral regurgitation. Cardiothoracic examination revealed bilateral diffuse rales, metallic first heart sound and a loud holo systolic murmur in apex with radiation to axilla. Transesophageal echocardiography revealed a large echo lucent area representing a separate chamber in the lateral part of left atrium which communicates with left ventricle through a relatively large orifice far from normal functioning prosthesis. In operation left atrial dissection was confirmed but the patient did not wean from cardiopulmonary bypass and died on the operating table.

Rapid progression of aortic wall vegetation.

The patient was a 28-year-old man referred to our center because of presence of a cardiac murmur and persistence of fever for 3 weeks. A harsh, loud systolic murmur in the left sternal border without radiation to the neck and an early diastolic murmur in the right second intercostal space could be heard. Transesophageal echocardiography revealed a subaortic web with moderate to severe aortic regurgitation and a large vegetation on the sinotubular junction of the aorta. Repetitive echocardiography showed the progression of the infective process to abscess and finally saccular aneurysm formation. The fibrous web was excised; the incompetent aortic valve and the inflamed aortic wall were replaced. To the best of our knowledge, this is the first report of a large vegetation in the ascending aorta at the site of a turbulent jet caused by an untreated subaortic web detected with transesophageal echocardiography. Our case illustrates the value of repetition of this technique in the diagnosis of complications of vegetation especially when situated in unusual sites.

Flash pulmonary edema heralding renal artery spasm.

Flash pulmonary edema is a condition characterized by sudden and recurrent episodes of dyspnea resulting from acute pulmonary venous congestion in the presence of normal or well-preserved left ventricular systolic function. This is usually associated with bilateral renal artery stenosis or stenosis of a single surviving kidney. We describe a patient with clinical presentation of flash pulmonary edema due to renal artery spasm. To the best of our knowledge, this is the first reported case of flash pulmonary edema due to renal artery spasm.

Prediction of intrapulmonary right to left shunt with left atrial size in patients with liver cirrhosis.

BACKGROUND: We screened a large number of liver transplant candidates with contrast echocardiography to detect intrapulmonary right to left shunt (IPS). We found that IPS is frequently present in patients with left atrial enlargement. This finding raises a question concerning the correlation between IPS and left atrial size in patients with liver cirrhosis. OBJECTIVES: The aim of this prospective study was to evaluate the possible correlation between left atrial size and IPS in patients with liver cirrhosis. METHODS: Adult patients (>18 years old) with documented liver cirrhosis underwent trans-thoracic contrast echocardiography with agitated saline. Left atrial dimension was measured by M-mode echocardiography. Stroke volume was calculated using left ventricular M-mode echocardiography. Patients with atrial fibrillation, intracardiac shunt, congenital heart defects, valvular heart disease and diastolic dysfunction were excluded. RESULTS: A total of 92 patients met all study criteria. Of these, 39 (42.3%) had IPS. Cardiac output was significantly greater in patients with IPS compared with those without IPS (5.68 +/- 0.83 L/min vs 4.75 +/- 0.76 L/min, P < 0.01). In a multi-variable model, after adjustment for body surface area and body mass index, left atrial enlargement was the strong predictor of IPS (area under the curve = 0.66) but when controlling for cardiac output, left atrial size was not an independent predictor of IPS. CONCLUSION: In the context of liver cirrhosis, patients with IPS have greater cardiac output compared with those without shunt. Left atrial enlargement, which reflects one aspect of increased cardiac output, is an indirect marker of IPS and greater left atrial dimension is associated with the presence of intrapulmonary right-to-left shunt.