Body mass index and age are associated with ventricular end-diastolic pressure in adults with a Fontan circulation.

INTRODUCTION: Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population. METHODS: All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analysed to identify variables associated with end-diastolic pressure. RESULTS: Forty-two patients were included. Median age was 24.0 years (20.9-29.0) with a body mass index of 23.7 kg/m2 (21.5-29.7). 10 (23.8%) patients had a systemic right ventricle. The median (Interquartile range) and mean pulmonary artery pressure were 11.0 mmHg (9.0-12.0) and 16.0 mmHg (13.0-18.0), respectively. On univariate analysis, end-diastolic pressure was positively associated with body mass index (p < 0.01), age > 25 years (p = 0.04), symptoms of heart failure (p < 0.01), systemic ventricular systolic pressure (p = 0.03), pulmonary artery mean pressure (p < 0.01), and taking diuretics (p < 0.01) or sildenafil (p < 0.01). End-diastolic pressure was negatively associated with aortic saturation (p < 0.01). On multivariate analysis, end-diastolic pressure was positively associated with age ≥ 25 years (p < 0.01), and body mass index (p = 0.04). CONCLUSIONS: In a cohort of adult patients with Fontan circulation undergoing catheterisation, end-diastolic pressure was positively associated with age ≥ 25 years and body mass index on multivariate analysis. Maintaining a healthy body mass index may offer haemodynamic benefit in adults with Fontan physiology.

Nonexercise activity thermogenesis in obesity management.

Obesity is linked to cardiovascular disease. The global increase in sedentary lifestyle is an important factor contributing to the rising prevalence of the obesity epidemic. Traditionally, counseling has focused on moderate- to vigorous-intensity exercise, with disappointing results. Nonexercise activity thermogenesis (NEAT) is an important component of daily energy expenditure. It represents the common daily activities, such as fidgeting, walking, and standing. These high-effect NEAT movements could result in up to an extra 2000 kcal of expenditure per day beyond the basal metabolic rate, depending on body weight and level of activity. Implementing NEAT during leisure-time and occupational activities could be essential to maintaining a negative energy balance. NEAT can be applied by being upright, ambulating, and redesigning workplace and leisure-time environments to promote NEAT. The benefits of NEAT include not only the extra calories expended but also the reduced occurrence of the metabolic syndrome, cardiovascular events, and all-cause mortality. We believe that to overcome the obesity epidemic and its adverse cardiovascular consequences, NEAT should be part of the current medical recommendations. The content of this review is based on a literature search of PubMed and the Google search engine between January 1, 1960, and October 1, 2014, using the search terms physical activity, obesity, energy expenditure, nonexercise activity thermogenesis, and NEAT.

Hypoplasia, pseudocoarctation and coarctation of the aorta - a systematic review.

Aortic arch abnormalities are uncommon and may be seen in association with other congenital cardiac anomalies. Coarctation, pseudocoarctation and hypoplastic aortic arch are known aortic arch abnormalities, with the former being well studied, whilst for the latter two, much less is known. There are similarities and differences that are important to distinguish among these three conditions in order to avoid errors in diagnosis that may result in unnecessary investigations, which may in turn result in physical or emotional harm to the patient. For this reason, we present a systematic review of the published literature providing an evidence-based overview that may be helpful to clinicians when faced with this diagnostic dilemma.

Papillary fibroelastoma of the pulmonary valve--a systematic review.

The pulmonary valve is the least affected site for valvular papillary fibroelastoma. With increasing use of routine echocardiography and other modalities of imaging, pulmonary valve papillary fibroelastomas (PVPFE) are being recognized more frequently. PVPFE is more often an incidental diagnosis and symptomatic patients usually present with shortness of breath. Embolic phenomena and right ventricular outflow tract obstruction are the most serious complications of PVPFE. Since PVPFE is rare, the purpose of this systematic review is to address demographic characteristics, the clinical presentation, management, and outcome of this benign tumor of the pulmonary valve.

Interferon-α and pericardial injury: a case report and literature review.

Interferon- α (IFN-α) alone or in combination with other chemotherapeutic agents has been used in the management of many malignant and non-malignant conditions. Pericarditis with or without pericardial effusion has been reported with IFN-α therapy, and available literature is limited to case reports. Pericardial constriction after interferon use has not been described in the published literature to date. We performed a systematic review of literature to address the demographic features, clinical presentation, diagnosis, treatment and outcome of interferon-related pericardial injury.

Faringocele / Pharyngocele

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Coronary artery ectasia in an adult Noonan syndrome detected on coronary CT angiography.

Coronary ectasia is rare in patients with Noonan syndrome. When suspected during echocardiography more common causes including Kawasaki disease in children and atherosclerosis coronary artery disease in adults should be ruled out. Coronary CT angiogram, a non-invasive imaging tool may be preferred over conventional coronary angiogram in the initial diagnosis and monitoring the progression of coronary ectasia in such patients. Aspirin may be considered to prevent coronary thrombosis.

Multicentric recurrent parotid pleomorphic adenoma in a child

Mixed tumours of the parotid gland are rare in childhood and recurrence of this tumour is infrequent. Some authors report a higher rate of recurrence with some histological subtypes, like hypocellular variant. Female sex and young age at initial treatment are also risk factor for recurrence. Also the first surgical treatment, tumoure nucleation or parotidectomy, has been implicated as a cause for recurrence. We present a case of a multicentric doubly recurrent parotid pleomorphic adenoma, 7 and 14 years after tumour enucleation, in a 9-year-old child. All the nodules resected showed the hypocellular variant of pleomorphic adenoma. We consider the relationships between the choice of treatment, the histologic subtypes of pleomorphic adenomas and multifocal tumours and recurrence. In this case, we believe the recurrence was related to surgical enucleation previously performed and the hypocellular histological subtype. We conclude that tumour enucleation is a risk factor for recurrence and total parotidectomy is the treatment of choice for pleomorphic parotid adenoma also in childhood (AU)

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Wegener´s granulomatosis: Description of a case with oral manifestation

Wegener’s granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener’s granulomatosis with oral manifestation, which began as chronic mastitis (AU)

Multicentric recurrent parotid pleomorphic adenoma in a child.

Mixed tumours of the parotid gland are rare in childhood and recurrence of this tumour is infrequent. Some authors report a higher rate of recurrence with some histological subtypes, like hypocellular variant. Female sex and young age at initial treatment are also risk factor for recurrence. Also the first surgical treatment, tumour enucleation or parotidectomy, has been implicated as a cause for recurrence. We present a case of a multicentric doubly recurrent parotid pleomorphic adenoma, 7 and 14 years after tumour enucleation, in a 9-year-old child. All the nodules resected showed the hypocellular variant of pleomorphic adenoma. We consider the relationships between the choice of treatment, the histologic subtypes of pleomorphic adenomas and multifocal tumours and recurrence. In this case, we believe the recurrence was related to surgical enucleation previously performed and the hypocellular histological subtype. We conclude that tumour enucleation is a risk factor for recurrence and total parotidectomy is the treatment of choice for pleomorphic parotid adenoma also in childhood.

Wegener's granulomatosis: description of a case with oral manifestation.

Wegener's granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener's granulomatosis with oral manifestation, which began as chronic mastitis.

Síndrome estilohioidal / Stylohyoid syndrome

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