Clinical implications of partial anomalous pulmonary venous connection: a rare cause of severe pulmonary arterial hypertension.

Isolated partial anomalous pulmonary venous connection (PAPVC) is an uncommon congenital heart anomaly that is sporadically associated with pulmonary arterial hypertension in the adult population. The diagnosis and therapy for this condition are challenging. We report on three cases of patients with unexpected severe precapillary pulmonary hypertension in single PAPVC treated with an upfront pulmonary arterial hypertension-specific combination therapy. Our cases indicate that the combination of PAPVC and pulmonary comorbidities may trigger the development of severe pulmonary arterial hypertension. The initiation of pulmonary arterial hypertension-targeted combination therapy revealed to be a safe and efficacious strategy for patients with PAPVC-associated severe pulmonary arterial hypertension.

Incidental finding of an absent left common carotid artery.

We describe a case of a young woman evaluated for Raynaud's phenomenon in whom an extremely rare variation, the absence of the left common carotid artery, was incidentally detected as an isolated finding. The detection of vascular anomalies may be important for future endovascular or surgical interventions.

Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: a case report.

Catastrophic antiphospholipid syndrome (CAPS) is a rare but devastating complication in patients with antiphospholipid syndrome (APS) with a high morbidity and mortality.We describe a case of a 30-year old female patient with immunoglobulin A (IgA) deficiency who underwent splenectomy because of idiopathic thrombocytopenic thrombocytopenia. Subsequently, an APS and finally systemic lupus erythematosus was diagnosed. After an uncomplicated pregnancy that was terminated by cesarean section, the patient developed severe CAPS with cerebral, myocardial, renal, and pulmonary involvement.Because of IgA deficiency, standard therapy consisting of plasmapheresis and intravenous immunoglobulins in addition to steroids was not tolerated. After 8 sessions of immunoadsorption (IAS), massive pulmonary hemorrhage was controlled but relapsed twice whenever IAS was terminated. As other immunosuppressive agents were considered dangerous because of the risk of infections in the face of severe hypogammaglobulinemia, we administered eculizumab, an inhibitor of the terminal complement pathway, which led to a persistent control of her disease. Interestingly, eculizumab therapy was associated with a further decline of complement C3 and C4 serum levels. The patient developed a subsequent flare of her systemic lupus erythematosus, potentially indicating that complement inhibition by eculizumab is not effective in preventing lupus flares.Taken together, we describe a unique case of life-threatening and difficult-to-treat CAPS with a good clinical response after terminal complement complex inhibition with eculizumab. Further controlled trials are necessary to investigate the value of eculizumab in patients with CAPS.

Comparison of image quality and radiation dose of different pulmonary CTA protocols on a 128-slice CT: high-pitch dual source CT, dual energy CT and conventional spiral CT.

OBJECTIVES: To compare image quality and radiation dose of high-pitch dual-source computed tomography (DSCT), dual energy CT (DECT) and conventional single-source spiral CT (SCT) for pulmonary CT angiography (CTA) on a 128-slice CT system. METHODS: Pulmonary CTA was performed with five protocols: high-pitch DSCT (100 kV), high-pitch DSCT (120 kV), DECT (100/140 kV), SCT (100 kV), and SCT (120 kV). For each protocol, 30 sex, age, and body-mass-index (mean 25.3 kg/m(2)) matched patients were identified. Retrospectively, two observers subjectively assessed image quality, measured CT attenuation (HU±SD) at seven central and peripheral levels, and calculated signal-to-noise-ratio (SNR) and contrast-to-noise-ratio (CNR). Radiation exposure parameters (CTDIvol and DLP) were compared. RESULTS: Subjective image quality was rated good to excellent in >92% (>138/150) with an interobserver agreement of 91.4%. The five protocols did not significantly differ in image quality, neither by subjective, nor by objective measures (SNR, CNR). By contrast, radiation exposure differed between protocols: significant lower radiation was achieved by using high-pitch DSCT at 100 kV (p < 0.01 in all). Radiation exposure of DECT was in between SCT at 100 kV and 120 kV. CONCLUSIONS: SCT, high-pitch DSCT, and DECT protocols techniques result in similar subjective and objective image quality, but radiation exposure was significantly lower with high-pitch DSCT at 100 kV. KEY POINTS: New CT protocols show promising results in pulmonary embolism assessment. High-pitch dual-source CT (DSCT) at 100 kV provides radiation dose savings for pulmonary CTA. High-pitch DSCT at 100 kV maintains diagnostic image quality for pulmonary CTA. Dual energy CT uses more radiation but also provides lung perfusion evaluation. Whether the additional perfusion data is worth the extra radiation remains undetermined.

Clinical relevance and scope of accidental extracoronary findings in coronary computed tomography angiography: a cardiac versus thoracic FOV study.

OBJECTIVE: To assess the spectrum and clinical relevance of extracoronary findings in coronary CT angiography (CCTA), and to compare a small (cardiac) field of view (FOV) to a large (thoracic) FOV setting. MATERIAL AND METHODS: 1084 consecutive patients (mean 57 years) with low-to-intermediate risk of coronary artery disease were enrolled. 542 CCTA scans were interpreted with small FOV (160-190mm(2)) encompassing the cardiac region. In another 542 CCTA (patients matched for age and gender), read-out of an additional full FOV (>320mm(2)) covering the thorax was performed. Clinical relevance of extracoronary findings was considered as either "significant" or "non-significant". "Significant" findings were subclassified as either score 1: findings necessitating immediate therapeutic actions, or score 2: findings with undoubted clinical or prognostic relevance, requiring clinical awareness, follow-up or further investigations (non-urgent). "Non-significant" findings were assigned to either score 3: findings not requiring follow-up or further tests, or as score 4: irrelevant incidental findings. RESULTS: Significantly more patients with extracoronary findings were identified by using a full FOV with 43.2% (234/542) compared to a small FOV with 33.6% (182/542) (p=0.001). Similarly, a higher total number of extracoronary findings (n=394) was found on full FOV compared to small FOV (n=250) (p<0.001). The detection rate of clinically significant findings was higher by using full FOV compared to small FOV (25.6% versus 15.4%) (p<0.001), out of those 2.2% versus 1.8% of findings required immediate actions (score 1), and 23.4% versus 13.6% (p=0.0001), respectively were of clinical relevance (non-urgent, score 2). The rate of malign findings was 0.2%, and of acute pulmonary embolism 0.1%. More lung pathologies were observed by using full FOV compared to small FOV (22% versus 7%) (p<0.0001), and the detection rate of intrapulmonary nodules increased by 2.1%. Prevalence of aortic valve calcification (n=72) was 13.3%, out of those 7% had less than 2cm(2) aortic valve orifice area. CONCLUSIONS: The interpretation of extracoronary findings on CCTA scans is mandatory given high prevalence of clinically significant findings by using a full "thoracic" FOV.