Right ventricular outflow tract obstruction caused by a right coronary cusp prolapse: An unusual finding

Patients with large sub-pulmonic ventricular septal defect (VSD) present early as a results of their complications. Some present late, due to the restriction of VSD by the right coronary cusp (RCC) due to its prolapse. In this report, we present a rare case of sub-pulmonic VSD in a 33-year-old man who developed a sub-pulmonic stenosis due to the prolapse of the RCC into the right ventricular outflow tract.     

Unexpected mechanism of mitral regurgitation in a patient post ALCAPA repair: Added value of three-dimensional echocardiography

Accurate assessment of etiology of mitral regurgitation (MR) is one of the key steps in the decision-making process and further clinical management of patients with severe MR. Our clinical case illustrates the added value of three-dimensional echocardiography (3DE) in assessment of mitral valve morphology and identification of an unexpected mechanism of MR which was not previously diagnosed using conventional echocardiography. 3DE helped to choose appropriate management strategy in this patient.     

Diagnosing pulmonary arteriovenous malformations in the presence of atrial septal defect and anomalous pulmonary venous drainage—An imaging challenge

Saline contrast echocardiography is a simple and effective method to diagnose the presence and type of right-to-left shunt in patients with unexplained cyanosis. It is considered a very sensitive test to diagnose pulmonary arteriovenous malformations. Our patient presented with unexplained cyanosis and transthoracic echocardiography showed an atrial septal defect and anomalous pulmonary venous drainage of the right and left upper pulmonary veins to the superior venacava. We describe how we used saline contrast echocardiography to demonstrate the presence of pulmonary arteriovenous malformations even in the presence of atrial septal defect and anomalous pulmonary venous drainage.     

Supracardiac total anomalous pulmonary venous connection with cor triatriatum sinister: A rare diagnosis confirmed by saline contrast echocardiography

In cor triatriatum sinister, the left atrium is divided by a membrane into a proximal and a distal chamber. Usually proximal chamber receives all the pulmonary veins and drains through an opening in the dividing membrane into distal chamber, which empties into left ventricle through the mitral valve. Rarely, the two chambers lack a communication and there is associated total anomalous pulmonary venous connection (TAPVC). We report a 1-month-old infant with cyanosis and heart failure, who had cor triatriatum sinister associated with supracardiac TAPVC. The case is reported for rarity of the association with a focus on contrast echocardiographic imaging.     

Do Obese Individuals With Hypertension Have More Difficult‐to‐Control Blood Pressure and End Organ Damage Than Their Nonobese Counterparts?

The authors assessed whether individuals with elevated body mass index (BMI) and hypertension had more difficult‐to‐control blood pressure (BP) and more evidence of end organ damage using data collected prospectively over 11 years from a secondary care hypertension clinic. A total of 1114 individuals were divided by BMI criteria into normal (n=207), overweight (n=440), and obese (n=467). Mean daytime, nighttime, and 24‐hour systolic BP and diastolic BP were similar in all groups. There was less nocturnal dip in obese compared with overweight groups (P=.025). Individuals with a normal BMI were taking fewer antihypertensive medications than those in the obese group (P=.01). Individuals classified as obese had a higher left ventricular mass index than those with a normal BMI (female, P=.028; male, P<.001); this relationship remained after multivariate linear regression. Obese individuals with hypertension required more medication to achieve similar mean ambulatory BP values, had less nocturnal dip in BP, and had a higher prevalence of left ventricular hypertrophy. As such, obese patients are at potentially increased risk of cardiovascular events.     

Epstein-Barr virus lymphoproliferation after bone marrow transplantation

We review 15 cases of secondary B-cell lymphoproliferative disorders that occurred among 2,475 patients who received allogeneic bone marrow transplants (BMTs) at the Fred Hutchinson Cancer Research Center (Seattle) between 1969 and 1987. The histopathologic findings in 14 of the 15 patients spanned a wide spectrum of lymphoproliferative lesions. One patient had features characteristic of angioimmunoblastic lymphadenopathy. Epstein-Barr virus (EBV) genomic sequences were identified by Southern blot analysis in each of the 13 patients evaluated. Ten of the 12 lesions evaluated originated in donor cells. In two patients, who had mixed chimerism after transplantation, the lesions originated in host cells. The combined evidence from immunoglobulin light chain staining and the analysis of immunoglobulin heavy chain gene rearrangement indicated that the lesions in most patients represented polyclonal proliferations that gave rise to clonal subpopulations. The results indicate an overall actuarial incidence of 0.6% for this complication in BMT recipients. Anti-CD3 monoclonal antibody (MoAb) treatment of acute graft-v-host disease (GVHD) and T cell depletion of the donor marrow were statistically significant risk factors, and GVHD appeared to play a contributing role, particularly in the setting of human leukocyte antigen (HLA) disparity. Two patients had no identifiable risk factors. Prophylaxis or treatment with acyclovir had no detectable effect in the patients; all but two died with uncontrolled lymphoproliferation.