Martijn C. Post

Background: Pulmonary right-to-left shunts are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). Screening has long been performed with the 100% oxygen method, but transthoracic contrast echocardiography (TTCE) is currently advised as first-line screenings technique. Purpose: The present study determined the diagnostic accuracy of the 100% oxygen method in detecting pulmonary shunts, using TTCE as gold standard. Methods: We included 189 persons, referred for HHT screening between 2004 and 2010, who underwent pulmonary shunt measurement with both the 100% oxygen method and TTCE. A pulmonary shunt of >5% measured with the 100% oxygen method was considered pathological. A quantitative three-point grading scale was used to classify the pulmonary shunt on TTCE (grade 0, no microbubbles; 1, 100 microbubbles). Results: A pulmonary shunt on TTCE was present in 95 out of 189 patients (50.3%). The 100% oxygen method demonstrated an abnormal shunt in only 49 (51.6%) of these patients (sensitivity 51.6%, specificity 87.2%, PPV 80.3, NPV 64.0%). Using the 100% oxygen method, a pathological pulmonary shunt was identified in 14.3%, 20% and 72.9% of patients with a pulmonary shunt grade 1, 2 and 3 on TTCE respectively. The 100% oxygen method indicated an abnormal pulmonary shunt in 12.8% of persons without any shunt on TTCE. In 13.3% of patients who underwent endovascular transcatheter embolotherapy of pulmonary shunts, the 100% oxygen method could not detect a pathological shunt. Conclusions: Large pulmonary shunts on TTCE may remain undetected using the 100% oxygen method. Our study confirms that the 100% oxygen method is not a useful screenings technique for the detection of pulmonary shunts.

We report a case where real-time three-dimensional transoesophageal echocardiography (3D-TEE) was able to detect a complicated transseptal puncture during a Mitraclip procedure which was not seen on the two-dimensional TEE (2D-TEE) images. This case demonstrates the incremental value of 3D-TEE during transseptal puncture.

Churg-Strauss syndrome or allergic angiitis and granulomatosis is a rare systemic vasculitis. Cardiac involvement is the leading cause of mortality and includes eosinophilic endomyocarditis. We present a case of complete resolution of subendocardial involvement after high-dose corticosteroids, diagnosed by contrast-enhanced cardiovascular magnetic resonance imaging.

We investigated the haemodynamic effect of percutaneous closure of an intra-atrial shunt, using non-invasive finger pressure measurements. Percutaneous closure of both patent foramen ovale (PFO) and atrial septal defect (ASD) is widely practised. Currently no data are available on short-term haemodynamic changes induced by closure. Twenty-five consecutive patients (mean age 49 +/- 17 years, 10 men) who underwent a percutaneous closure of a PFO (n = 15) or ASD (n = 10) were included in this study. During the procedure blood pressure and heart rate (HR) were monitored continuously with a Finometer. Changes in systolic, mean, and diastolic pressure, stroke volume (SV), cardiac output (CO) and total peripheral resistance (TPR) were computed from the pressure registrations using Modelflow methodology. Baseline characteristics were similar for the PFO and ASD patients. After PFO closure none of the haemodynamic parameters changed significantly. After ASD closure the systolic, mean, and diastolic pressures increased 7.1 +/- 5.4 (P = 0.003), 3.8 +/- 3.5 (P = 0.007) and 2.0 +/- 3.0 mmHg (P = ns) respectively. HR decreased 5.1 +/- 5.3 beats per minute (P = 0.01). SV, CO and TPR increased 8.5 +/- 6.4 ml (13.5%; P = 0.002), 0.21 +/- 0.45 l min(-1) (5.6%; P = ns) and 0.02 +/- 0.14 dynes (4.1%; P = ns) respectively. The changes in SV differ between the PFO and ASD patients (P = 0.009). Using non-invasive finger pressure measurements, we found that SV, mean and systolic blood pressure increased immediately after percutaneous closure of an ASD in adults, whereas the percutaneous PFO closure had no effect on haemodynamic characteristics.

A 62-year-old woman with a history of transcatheter atrial septal defect (ASD) closure using a Cardioseal/Starflex® device was admitted for recurrent transient ischaemic attacks 3 years after closure. A transoesophageal echocardiogram (TEE) revealed a large, mobile, thrombus-like structure attached to the left-atrial side of the device as a potential cardio-embolic source. There was no evidence of device malposition or arm fractures. Four months after initiation of oral anticoagulants, a TEE showed no resolution of the thrombus-like structure. The patient refused the recommended surgical exploration. Unfortunately, she was re-admitted for recurrent stroke 2 months later. Finally, she approved with exploration through minimal invasive port access surgery. During surgery, a structure of 2 cm covering the device was removed. The device was explanted, followed by primary ASD closure. Microscopic examination confirmed the structure to be a fresh fibrin thrombus with granulocytes. The patient recovered well. Treatment with oral anticoagulants was continued for at least 6 months.