Modes of death and clinical outcomes in adult patients with hypertrophic cardiomyopathy in Thailand.

BACKGROUND: There are limited data about modes of death and major adverse cardiovascular events (MACEs) in patients with hypertrophic cardiomyopathy (HCM) in South East Asian population. The aim of the study was to examine modes of death and clinical outcomes in Thai patients with HCM. METHODS: Between January 1, 2009 and December 31, 2013, 166 consecutive patients with HCM diagnosed in our institution were evaluated. Five patients were excluded because of non-Thai ethnic groups (n = 3) and diagnosis of myocardial infarction at initial presentation documented by coronary angiography (n = 2). The final study population consisted of 161 patients with HCM. HCM-related deaths included: (1) sudden cardiac death (SCD) - death due to sudden cardiac arrest or unexpected sudden death; (2) heart failure - death due to refractory heart failure; or (3) stroke - death due to embolic stroke associated with atrial fibrillation. MACEs included: (1) SCD, sudden unexpected aborted cardiac arrest, fatal, or nonfatal ventricular arrhythmia (ventricular fibrillation or sustained ventricular tachycardia); (2) heart failure (fatal or non-fatal), or heart transplantation; or (3) stroke - fatal or non-fatal embolic stroke associated with atrial fibrillation. RESULTS: One hundred and sixty-one Thai patients with HCM (age 66 ± 16 years, 58% female) were enrolled. Forty-two patients (26%) died over a median follow-up period of 6.8 years including 25 patients (16%) with HCM-related deaths (2%/year). The HCM-related deaths included: heart failure (52% of HCM-related deaths; n = 13), SCD (44% of HCM-related deaths; n = 11), and stroke (4% of HCM-related deaths, n = 1). The SCDs occurred in 6.8% of patients (1%/year). Eighty-four major MACEs occurred in 65 patients (41, 5%/year). The MACEs included: 40 heart failures in which 2 patients underwent heart transplants; 22 SCDs and nonfatal ventricular arrhythmias; and 22 fatal or nonfatal strokes. CONCLUSIONS: The most common mode of death in adult patients with HCM in Thailand was heart failure followed by SCD. About one-third of the patients experiencing heart failure died during the 6.8 years of follow-up. SCDs occurred in 7% of patients (1%/year), predominantly in the fourth decade or later.

High dose oral vitamin C and mesenchymal stem cells aid wound healing in a diabetic mouse model.

OBJECTIVE: This study sought to determine the effects of oral vitamin C (VitC) and mesenchymal stem cells (MSCs) on wound healing in diabetic nude mice. METHOD: Bilateral, full-skin thickness wounds were created as an in vivo wound model in BALB/C diabetic nude mice. The mice were separated into five groups: control (CON); diabetes mellitus (DM, from a streptozotocin injection); DM treated with MSCs (DM+MSCs); DM treated with VitC (DM+VitC), and DM treated with MSCs and VitC (DM+MSCs+VitC). After wounding, daily oral-feeding of high dose VitC (1.5g/l) was administered, and a single dose of MSCs (1x106 cells) was given topically using matrix gel application to the wounded area. RESULTS: At day seven, the lowest rate of wound healing, in terms of percentage of wound closure, appeared in the DM group, as compared with the CON and all other treatment groups (mean percentage of wound closure and standard deviation), CON=75.94±7.09%; DM=55.65±9.59%; DM+MSCs=78.57±6.46%; DM+VitC=77.52±3.31%; and DM+MSCs+VitC=84.61±2.87%, p≤0.05. At day 14 post-wounding, the combination of oral high dose VitC and MSCs accelerated wound healing (91.44±3.19%, p≤0.05). In addition, the highest capillary density in DM+MSCs+VitC was obtained at 14 days post-wounding (29.49±7.30%, p≤0.05). CONCLUSION: The findings of this study highlight the possibility of using oral high dose VitC in adjunct to MSCs to increase angiogenesis and accelerate diabetic wound healing in an animal model. This novel therapeutic approach should be studied further to test if it could be a useful adjunct of existing therapies to prevent infection and amputation in patients with diabetes.

Extensive loculated ascites in hepatic amyloidosis.

CONTEXT: Amyloidosis is a disease of extracellular deposition of misfolded proteinaceous subunits, which could be systemic or localized disease. Though hepatic amyloidosis was not uncommon in autopsy series, most cases of hepatic amyloidosis were asymptomatic. Ascites, jaundice, portal hypertension, and gastrointestinal bleeding from esophageal varices were reported in literature. CASE REPORT: A 42-year-old man with end-stage renal disease on hemodialysis and recent small bowel obstruction presented with chronic abdominal pain. Computed tomography of abdomen and pelvis showed extensive loculated ascites and multiple small bowel loops tethered to adhesions and hepatomegaly. Finally, hepatic venography and liver biopsy confirmed hepatic amyloidosis with portal hypertension. The patient was waiting for liver transplant for definite treatment. CONCLUSION: We report a rare case of hepatic amyloidosis with prior small bowel obstruction presented with extensive loculated ascites and multiple small bowel loops tethered to adhesions.

STEMI complicated with serum sodium of 113 mmol/L from syndrome of inappropriate antidiuretic hormone secretion: How worse could it be?

Hyponatremia commonly occurs in acute coronary syndrome and has been recognized as a worse prognostic indicator in patients with ST-segment elevation myocardial infarction (STEMI). However STEMI with preexisting hyponatremia from syndrome of inappropriate antidiuretic hormone secretion (SIADH) has never been described in the literature. We describe a case of 59-year-old woman who presented with STEMI and received emergent percutaneous coronary intervention who also had SIADH with the lowest serum sodium measurement of 113 mmol/L. Initially, she was treated with hypertonic saline to reduce central nervous system complications. Then, vasopressin receptor 2 antagonist and demeclocycline were started as well as fluid restriction and salt tablet. Her sodium level and clinical symptoms improved. Subsequently, we found cavitary right upper lung mass and a biopsy report revealed small cell lung cancer as a cause of SIADH. Severe hyponatremia from SIADH complicated with STEMI could potentially have reduced adverse outcomes by normalizing sodium level through vasopressin receptor 2 antagonist or demeclocycline. .

A rare complication of a common stress test.

In 2008, regadenoson, a selective adenosine2A (A2A) receptor agonist, was approved by the US Federal and Drug Administration for use as a pharmacologic stress agent in myocardial perfusion studies. By stimulating A2A receptors in coronary smooth muscle, it can increase coronary blood flow by 2.5-fold or greater. Previous data showed non-inferiority of regadenoson in detecting reversible myocardial ischemia, compared to adenosine. Given less serious adverse effects, being better tolerated and easily administered, regadenoson has been widely used for myocardial perfusion imaging. As adenosine receptors have many sub-types and are located in multi-organ systems, regadenoson can cause various adverse effects, including bronchospasm, atrioventricular block, or hypotension. However, adverse effects on the central nervous system are rarely reported. As adenosine receptors (A1 and A2A receptors) play a major role in neuron-glial cells interaction, regadenoson can provoke seizure through A2A receptor activation. We hereby report a case of regadenoson associated-seizure and review seizure mechanism. This may raise more concern for a rare serious adverse effect of regadenoson which should be taken into consideration when selecting cardiac stress modalities. .

Prognostic value of prolonged QTc interval in patients with acute pulmonary embolism.

BACKGROUND/OBJECTIVES: Acute pulmonary embolism (PE) can be a life-threatening condition. Right ventricular function evaluated by echocardiography, brain natriuretic peptide levels and several patterns on the electrocardiogram have been utilized to guide treatment and prognosis. Case reports described heart rate corrected QT (QTc) prolongation as an ECG finding associated with PE. However, the prognostic value of QTc prolongation has not been studied. METHODS: Retrospective chart review of 300 consecutive patients (mean age 60.3 ± 17.6 years; 40.7% men) diagnosed with acute PE by computed tomography pulmonary angiography or ventilation perfusion scan were studied. Patients were divided into two groups: a prolonged QTc group with QTc >460 milliseconds (n=178) and a control group (n=122). We retrospectively reviewed medical records, electrocardiography, echocardiography and radiography results. Statistical analyses included unpaired t-test and Fisher's exact test using Stata version12. RESULTS: The prolonged QTc group demonstrated significantly increased right ventricular dilatation and systolic dysfunction. Additionally, the duration of hospitalization and intensive care unit stay were longer in the prolonged QTc group. Further, the prolonged QTc group had more hypotensive episodes and received thrombolytic treatment more frequently. There was no statistical difference in in-patient mortality rates (4.5% for the study group and 4.2% for the control group, P=1). CONCLUSIONS: Prolonged QTc may prove a novel predictor for evaluating prognosis in acute PE. Larger studies will need to confirm this finding.

Concomitant typhlitis and Clostridium difficile colitis developed after first R-CHOP chemotherapy in a non-Hodgkin lymphoma patient.

Typhlitis or neutropenic enterocolitis (NEC) is a life-threatening condition that occurs in neutropenic patients. Early recognition is crucial owing to high death rate. We present a case of a 54-year-old man, diagnosed with non-Hodgkin lymphoma who received a first cycle of rituximab, cyclophosphamide, hydroxydaunorubicin (doxorubicin), oncovin (vincristine), prednisolone (R-CHOP) chemotherapy 10 days prior presenting. He developed fever, mucositis, watery diarrhoea and right lower quadrant pain with rebound tenderness. He also had neutropenia, with an absolute neutrophil count of zero. CT abdomen confirmed the diagnosis of typhlitis, demonstrating characteristic terminal ileum, caecal and right-sided colon involvement. Moreover, stool PCR was also positive for toxigenic Clostridium difficile. Therefore, the patient was diagnosed with concomitant typhlitis and C difficile-associated diarrhoea (CDAD). He was empirically treated with intravenous cefepime, intravenous metronidazole and oral vancomycin. His symptoms resolved in 10 days. This case illustrated a successful medical treatment of typhlitis in concomitance with CDAD.

Warburg effect associated with transformed lymphoplasmacytic lymphoma to diffuse large B-cell lymphoma.

Warburg effect is a rare metabolic complication in hematologic malignancies, commonly presented with lactic acidosis and hypoglycemia. Mechanism explained by abnormality of mitochondrial oxidative phosphorylation in cancer cells and energy production is mostly dependent on anaerobic respiration or glycolysis pathway to meet large tumor demand. We present a case with history of lymphoplasmacytic lymphoma and Waldenstörm macroglobulinemia, partial response to chemotherapy. Lymphoplasmacytic lymphoma transformed to diffuse large B-cell lymphoma, which is aggressive and rapid progression, leading to Warburg effect. Patient developed more than 10-cm retroperitoneal mass less than 1 year, and his symptoms were progressively worsening within 3 weeks. Warburg effect represents poor prognosis no matter with or without hypoglycemia. Treatment of choice is cytoreduction with early chemotherapy. Our patient died 2 days after Warburg effect occurred.