RESUMEN
Coronary cameral fistulas (CCFs) are rare and are characterized by an abnormal connection between a coronary artery and any of the four chambers of the heart. Most cases of CCFs are asymptomatic. The most common presentation in symptomatic patients includes chest pain or heart failure; however, arrhythmias are rarely associated. We report the case of a 32-year-old male previously unknown to have any medical illnesses. He presented to the clinic with complaints of frequent palpitations, necessitating recurrent admissions. His electrocardiograms revealed regular wide complex tachycardia with a right bundle branch block pattern, suggestive of fascicular ventricular tachycardia. During hospitalization, an elective coronary angiography showed a large CCF originating from the right posterior descending coronary artery and draining into the left ventricle. Moreover, cardiac magnetic resonance imaging did not show any scar or evidence of cardiomyopathies. The patient underwent a successful catheter-based right coronary artery to left ventricular fistula occlusion with coils. In addition, the patient underwent a complex electrophysiological study with three-dimensional mapping and ablation. The presented case underscores the rarity and complexity of such clinical presentations. It also highlights the importance of a multidisciplinary approach in addressing this unique cardiac anomaly.
RESUMEN
Background and objectives Obesity can increase cardiac mass and affect cardiac performance independently from other risk factors. Several studies have identified an association in patients who already have comorbidities, however, few studies focused on obesity as an isolated risk factor. This study aimed to assess the associations between isolated obesity and heart morphological and functional characteristics. Methods This was a cross-sectional study that recruited 114 patients referred for echocardiographic study in King Faisal Cardiac Center. Adult patients who had a body mass index (BMI) above 25 kg/m2 were included, while patients with comorbidities such as hypertension, diabetes mellitus, dyslipidemia, or those who use medications for chronic diseases were excluded from this study. Variables of interest that we collected were age, gender, weight, BMI, and those related to morphological and functional changes in the heart including left ventricular mass index (LVMI), LV end-diastolic volume, and left ventricular ejection fraction (LVEF). Results Most of the study participants (63.8%) were class II or class III obesity and about 80% were males. The mean ± SD of LVEF was 55.7% ± 2.8%, while the mean of the left ventricular mass index was 28.5±5.84. The mean of LV end-diastolic volume index (LVEDVI) was slightly higher among males than females (48.8±11.6 versus 46.4±11.7 ml/m2), however, this difference was not statistically significant (p-value= 0.395). There was no correlation between BMI and LVMI in females (R - 0.226, R2 0.05, P-value 0.37), while the LVMI was found to have a negative correlation between BMI and male gender that was significant (R - 0.292, R2 0.09, P-value 0.0052). It was found that there is no correlation between LVEF and BMI for males and females (male= R 0.093, R2 0.032, P-value 0.093; female= R 0.172, R2 0.029, P-value 0.434). With regards to the LVEDVI, there was a negative correlation between higher BMI and male gender that was significant (male= R - 0.396, R2 0.157, P-value 0.0001) while it was not significant in females (R -0.0298, R2 0.0009, P-value 0.893). Conclusions We have found that cardiac function is not affected by isolated obesity. However, indexed cardiac parameters like LVM and LV end diastolic volume were negatively correlated with higher BMI and positively correlated with relative wall thickness (RWT) only in males. This negative correlation might be one of the triggers to the development of obesity-induced cardiomyopathy.
RESUMEN
Papillon-Lefèvre syndrome (PLS) is a rare genetic disease that causes dermatological and dental symptoms that usually start from early age. Dermatological findings include hyperkeratoderma over the palms and soles that are usually thought of as persistent psoriasis at first. Dental findings include severe caries in the teeth that lead to premature dental loss. We present a case of an otherwise healthy seven-year-old child with classical presentation of PLS with both dermatological and dental findings. He first presented to the dermatology clinic when he was five years old brought by his parents complaining of dry scaly patches on the palm of the hands and soles of the feet. On further history it was found that he is a child of first-degree consanguinity, and he had these patches since he was four months old. On examination, he was found to have an erythematous hyperkeratotic skin plaques and papules with scales over the planter and palmar aspect of both hands with similar lesions observed on both feet, legs, scalp, and ears with nail pitting. The diagnosis of PLS was confirmed by whole-exome sequencing (WES) and the patient was started on acitretin capsules and started to show improvement.