Search

BACKGROUND Kommerell diverticulum with a right-sided aortic arch is a rare congenital anatomical condition most often observed in adults. A possible etiology of the subclavian artery's anomalous origin would be an abnormality in regression of the fourth primitive aortic arch during embryonic development. CASE REPORT We report on the case of a 16-year-old female patient presenting with complaints of occasional tachycardia and mild non-specific dyspnea after anxiety crises. Physical examination revealed lower amplitude of the pulses in the left upper limb compared to the right upper limb, and difference in blood pressure (BP) values of 80×60 mmHg, and 100×60 mmHg, respectively. Different radiological imaging modalities were performed to elucidate a possible vascular abnormality. Multislice detector computed tomography angiography of the thoracic aorta and supra-aortic trunks showed a right-sided aortic arch and an aberrant origin of the left subclavian artery with a retroesophageal course and dilation of its emergence (Kommerell diverticulum), as well as duplicity of the right vertebral artery (RVA). Considering the actual small diameter of the diverticulum and the absence of dysphagia or severe external esophageal compression analyzed by the esophagogram, vascular surgery was not indicated. Since complications have been described in the literature, the patient must be kept under observation in the future. CONCLUSIONS Congenital vascular alterations, including Kommerell diverticulum with right-sided aortic arch and the aberrant origin of the left subclavian artery, should be suspected in otherwise asymptomatic young patients with few clinical manifestations. Investigation with different imaging methods helps to clarify the vascular abnormalities, to support a possible surgical procedure indication, and to monitor the patients in follow-up.

Myocardial iron quantification remains limited to 1.5 T systems with T2* measurement. The present study aimed at comparing myocardial T2* values at 1.5 T to T1 and T2 mapping at 3.0 T in patients with iron overload and healthy controls. A total of 17 normal volunteers and seven patients with a history of myocardial iron overload were prospectively enrolled. Mid-interventricular septum T2*, native T1 and T2 times were quantified on the same day, using a multi-echo gradient-echo sequence at 1.5 T and T1 and T2 mapping sequences at 3.0 T, respectively. Subjects with myocardial iron overload (T2* < 20 ms) in comparison with those without had significantly lower mean myocardial T1 times (868.9 ± 120.2 vs. 1170.3 ± 25.0 ms P = 0.005 respectively) and T2 times (34.9 ± 4.7 vs. 45.1 ± 2.0 ms P = 0.007 respectively). 3 T T1 and T2 times strongly correlated with 1.5 T, T2* times (Pearson's r = 0.95 and 0.91 respectively). T1 and T2 measures presented less variability than T2* in inter- and intra-observer analysis. Native myocardial T1 and T2 times at 3 T correlate closely with T2* times at 1.5 T and may be useful for myocardial iron overload quantification.

Background: Pulmonary arterial hypertension is a severe and progressive disease. Its early diagnosis is the greatest clinical challenge., Objective: To evaluate the presence and extension of the delayed myocardial contrast-enhanced cardiovascular magnetic resonance, as well as to verify if the percentage of the myocardial fibrosis mass is a severity predictor., Methods: Cross-sectional study with 30 patients with pulmonary arterial hypertension of groups I and IV, subjected to clinical, functional and hemodynamic evaluation, and to cardiac magnetic resonance., Results: The mean age of patients was 52 years old, with female predominance (77%). Among the patients, 53% had right ventricular failure at diagnosis, and 90% were in functional class II/III. The mean of the 6-minute walk test was 395 m. In hemodynamic study with right catheterism, the mean average pulmonary arterial pressure was 53.3 mmHg, of the cardiac index of 2.1 L/min.m(2), and median right atrial pressure was 13.5 mmHg. Delayed myocardial contrast-enhanced cardiovascular magnetic resonance was found in 28 patients. The mean fibrosis mass was 9.9 g and the median percentage of fibrosis mass was 6.17%. The presence of functional class IV, right ventricular failure at diagnosis, 6-minute walk test < 300 meters and right atrial pressure > 15 mmHg, with cardiac index < 2.0 L/min.m(2), there was a relevant association with the increased percentage of myocardial fibrosis., Conclusion: The percentage of the myocardial fibrosis mass indicates a non-invasive marker with promising perspectives in identifying patients with high risk factors for pulmonary hypertension.

Background: Patients with Sickle cell disease (SCD) who receive regular transfusions are at risk for developing cardiac toxicity from iron overload. The aim of this study was to assess right and left cardiac volumes and function, late gadolinium enhancement (LGE) and iron deposits in patients with SCD using CMR, correlating these values with transfusion burden, ferritin and hemoglobin levels., Methods: Thirty patients with SCD older than 20 years of age were studied in a 1.5 T scanner and compared to age- and sex-matched normal controls. Patients underwent analysis of biventricular volumes and function, LGE and T2* assessment of the liver and heart., Results: When compared to controls, patients with SCD presented higher left ventricular (LV) volumes with decreased ejection fraction (EF) with an increase in stroke volume (SV) and LV hypertrophy. The right ventricle (RV) also presented with a decreased EF and hypertrophy, with an increased end-systolic volume. Although twenty-six patients had increased liver iron concentrations (median liver iron concentration value was 11.83 ± 9.66 mg/g), only one patient demonstrated an abnormal heart T2* < 20 msec. Only four patients (13%) LGE, with only one patient with an ischemic pattern., Conclusions: Abnormal heart iron levels and myocardial scars are not a common finding in SCD despite increased liver iron overload. The significantly different ventricular function seen in SCD compared to normal suggests the changes in RV and LV function may not be due to the anemia alone. Future studies are necessary to confirm this association.

Thalassemia major (TM) patients have altered ventricular volumes and ejection fraction compared to normals, although evidence for these findings stem from restricted patient groups and has never been reproduced. We sought to evaluate cardiac parameters by cardiovascular magnetic resonance (CMR) in a group of young TM patients not covered by previous studies that are more representative of the TM population in many countries. Seventy patients including 40 TM with normal myocardial iron concentrations, and 30 age- and gender-matched normal (NL) volunteers underwent a CMR study for assessment of left and right ventricle volumes and function using a 1.5-T scanner. Left and right ventricle ejection fraction, indexed systolic and diastolic volumes, and indexed mass were compared between the two groups. Mean age of TM patients was 18.2 ± 7.1 versus 17.5 ± 8.5 years in NL with no significant differences (P = 0.73). There was no difference in left ventricular (LV) ejection fraction between the groups (TM 64.9 ± 5.7 %, NL 64.9 ± 5.2 %; P = 0.97). LV normalized end-diastolic and end-systolic volumes were significantly higher in patients with TM compared to NL volunteers (76.8 ± 19.4 versus 66.6 ± 11.7 mL/m², P = 0.008, and 27.0 ± 8.8 versus 23.6 ± 5.0 mL/m², P = 0.045). LV indexed mass was also higher in TM patients compared to NL (51.2 ± 11.9 versus 42.0 ± 8.5 g/m², P < 0.001). No significant differences were observed in right ventricular parameters. In conclusion, younger patients with TM do not present different left or right ventricular function values compared to normal controls despite having increased left ventricular volumes and mass.

Pulmonary hypertension (PH) is a progressive disease that leads to substantial morbidity and eventual death. Pulmonary multidetector CT angiography (MDCTA), pulmonary MR angiography (MRA) and MR-derived pulmonary perfusion (MRPP) imaging are non-invasive imaging techniques for the differential diagnosis of PH. MDCTA is considered the gold standard for the diagnosis of pulmonary embolism, one of the most common causes of PH. MRA and MRPP are promising techniques that do not require the use of ionising radiation or iodinated contrast material, and can be useful for patients for whom such material cannot be used. This review compares the imaging aspects of pulmonary MRA and 64-row MDCTA in patients with chronic thromboembolic or idiopathic PH.

In this study, we evaluated patients with pulmonary arterial hypertension (PAH) and impaired right ventricular function. We used cardiac MRI for the detection of myocardial delayed enhancement (MDE) and its possible association with other clinical variables. 20 patients (6 males and 14 females, aged 44.5+/-11 years; 15 New York Heart Association class III, 5 class IV) with known PAH (13 idiopathic, 7 resulting from chronic pulmonary embolism) were evaluated for the detection of MDE. Short-axis cine images of the heart were made for ventricular function assessment using a steady-state free precession sequence. For MDE evaluation, a short-axis phase-sensitive inversion recovery sequence was performed 10 min after intravenous administration of 0.2 mmol kg(-1) gadodiamide. Right ventricle (RV) systolic dysfunction, RV enlargement and RV hypertrophy were present in 20 patients (RV ejection fraction, 21.5+/-7.2%; RV diastolic diameter, 5.97+/-0.79 cm; RV wall thickness, 0.73+/-0.10 cm). 13 of the 20 patients (65%) were positive for MDE (10 anterior, 12 inferior). All 13 positive patients with MDE demonstrated small hyperintense areas at the insertion points of the RV free wall in the interventricular septum. We found no significant correlation between MDE and ejection fraction or other haemodynamic variables. In this study, MDE correlated positively only with the duration of disease. We found that septal MDE can be present in patients with PAH and impaired ventricular function. However, further studies are necessary to investigate this possible association and its prognostic implication.

The purpose of this study is to report three cases of left ventricular myocardium non-compaction (LVNC), with emphasis on the MRI findings. From May 2006 to February 2007, three patients -- 2 females (6 years and 42 years of age) and 1 male (18 years of age) -- presented to our radiology department, two of them with fatigue, shortness of breath and episodes of syncope and arrhythmia, for further investigation by cardiac MRI because an apparent asymmetrical pattern of hypertrophy of the left ventricular myocardium was suspected by transthoracic echocardiography. The 18-year-old patient was only experiencing arrhythmia, and arrhythmogenic right ventricular dysplasia was suspected. The images (produced by a 1.5T MRI system) were interpreted by two experienced radiologists and post-processed with Argus software (Siemens, Germany) for ejection fraction calculation. In all three patients, MRI aided in the correct identification of prominent ventricular myocardial trabeculations and deep intertrabecular recesses communicating with the ventricular cavity, as well as areas of hypokinesia with depressed systolic function, and showed the absence of myocardial delayed enhancement and other structural heart defects. In conclusion, cardiac MRI was useful for correctly identifying this rare congenital heart disorder and appears to increase diagnostic accuracy. Although considered a rare anomaly, radiologists should be capable of recognizing LVNC, as current non-invasive imaging methods have increased the frequency of this diagnosis and timely detection is vital in considering early-stage transplantation.