Chronic thromboembolic pulmonary hypertension.

Chronic pulmonary thromboembolic disease is an important cause of severe pulmonary hypertension, and as such is associated with significant morbidity and mortality. The prognosis of this condition reflects the degree of associated right ventricular dysfunction, with predictable mortality related to the severity of the underlying pulmonary hypertension. Left untreated, the prognosis is poor. Pulmonary endarterectomy is the treatment of choice to relieve pulmonary artery obstruction in patients with chronic thromboembolic pulmonary hypertension and has been remarkably successful. Advances in surgical techniques along with the introduction of pulmonary hypertension-specific medication provide therapeutic options for the majority of patients afflicted with the disease. However, a substantial number of patients are not candidates for pulmonary endarterectomy due to either distal pulmonary vascular obstruction or significant comorbidities. Therefore, careful selection of surgical candidates in expert centres is paramount. The current review focuses on the diagnostic approach to chronic thromboembolic pulmonary hypertension and the available surgical and medical therapeutic options.

Patients with left bundle branch block pattern and high cardiac risk myocardial SPECT: does the current management suffice?

INTRODUCTION: Myocardial perfusion SPECT (MPS) is frequently used for cardiovascular risk stratification. The significance of MPS in patients with abnormal electrical ventricular activation is often questionable. This review assesses the value of MPS for risk stratification of patients with intrinsic left bundle branch block or that due to right ventricular apical pacing. METHODS: We reviewed the literature by a search of the MEDLINE database (January 1980 to September 2010). The terms prognosis or prognostic value were combined with SPECT and LBBB or pacing or pacemakers. MPS was categorised as low and high risk according to the original definitions. RESULTS: We identified 11 studies suitable for review. A low-risk MPS is associated with a low risk of cardiac events whereas high-risk MPS carries a 4.8-fold increased risk, 95% CI [3.2 - 7.2] (p < 0.0001). Despite secondary prevention and an improved medical and interventional care, these figures have hardly changed over time. CONCLUSION AND CLINICAL IMPLICATIONS: A low-risk MPS permits a policy of watchful waiting whereas a high-risk MPS requires further analysis and treatment. The persistent high cardiac death and acute myocardial infarction rate after a high-risk MPS suggest that the current management of these patients does not suffice and needs reconsideration.

Predicting 30-day mortality of aortic valve replacement by the AVR score.

OBJECTIVES: The objective of this study is to develop a simple risk score to predict 30-day mortality of aortic valve replacement (AVR). METHODS: In a development set of 673 consecutive patients who underwent AVR between 1990 and 1993, four independent predictors for 30-day mortality were identified: body mass index (BMI) ≥30, BMI <20, previous coronary artery bypass grafting (CABG) and recent myocardial infarction. Based on these predictors, a 30-day mortality risk score-the AVR score-was developed. The AVR score was validated on a validation set of 673 consecutive patients who underwent AVR almost two decennia later in the same hospital. RESULTS: Thirty-day mortality in the development set was ≤2% in the absence of any predictor (class I, low risk), 2-5% in the solitary presence of BMI ≥30 (class II, mild risk), 5-15% in the solitary presence of previous CABG or recent myocardial infarction (class III, moderate risk), and >15% in the solitary presence of BMI <20, or any combination of BMI ≥30, previous CABG or recent myocardial infarction (class IV, high risk). The AVR score correctly predicted 30-day mortality in the validation set: observed 30-day mortality in the validation set was 2.3% in 487 class I patients, 4.4% in 137 class II patients, 13.3% in 30 class III patients and 15.8% in 19 class IV patients. CONCLUSIONS: The AVR score is a simple risk score validated to predict 30-day mortality of AVR.

Percutaneous mitral valve repair using the edge-to-edge technique in a high-risk population.

Background. Percutaneous mitral valve (MV) repair using the edge-to-edge clip technique might be an alternative for patients with significant mitral regurgitation (MR) and an unacceptably high risk for operative repair or replacement. We report the short-term safety and efficacy of this new technique in a high-risk population.Methods. All consecutive high-risk patients who underwent percutaneous MV repair with the Mitraclip(®) between January and August 2009 were included. All complications related to the procedure were reported. Transthoracic echocardiography for MR grading and right ventricular systolic pressure (RVSP) measurement were performed before, and at three and 30 days after the procedure. Differences in NYHA functional class and quality of life (QoL) index were reported. Results. Nine patients were enrolled (78% male, age 75.9±9.0 years, logistic EuroSCORE 33.8±9.0%). One patient developed inguinal bleeding. In one patient partial clip detachment occurred, a second clip was placed successfully. The MR grade before repair was ≥3 in 100%, one month after repair a reduction in MR grade to ≤2 was present in 78% (p=0.001). RVSP decreased from 43.9±12.1 to 31.6±11.7 mmHg (p=0.009), NYHA functional class improved from median 3 (range 3 to 4) to 2 (range 1 to 4) (p=0.04), and QoL index improved from 62.9±16.3 to 49.9±30.7 (p=0.12). Conclusion. In high-risk patients, transcatheter MV repair seems to be safe and a reduction in MR can be achieved in most patients, resulting in a short-term improvement of functional capacity and QoL. (Neth Heart J 2010;18:437-43.).

Aortic aneurysm formation following coarctation repair by Dacron patch aortoplasty.

We describe the finding of an aortic aneurysm in an asymptomatic 43-year-old male, who was managed by Dacron patch aortoplasty for native coarctation of the aorta 25 years before. The role of magnetic resonance angiography as standard imaging technique in lifelong postoperative surveillance is discussed subsequently. (Neth Heart J 2010;18:376-7.).

Postural electrocardiographic abnormalities.

False-positive stress testing is a common problem in daily practice. We present a young male patient with pseudo-ischaemic ST-segment changes due to upright position. (Neth Heart J 2010;18:99-102.).

Long-term efficacy of bosentan in inoperable chronic thromboembolic pulmonary hypertension.

UNLABELLED: Background. Inoperable chronic thromboembolic pulmonary hypertension (CTEPH) is associated with a poor survival.Objectives. To evaluate the long-term response to a dual endothelin receptor antagonist in patients with inoperable CTEPH.Methods. All consecutive 18 patients (mean age 63+/-14 years) treated with bosentan for symptomatic inoperable CTEPH were included. Efficacy was evaluated by the log value of serum levels of N-terminal-pro brain natriuretic peptide (log NTpro BNP), New York Heart Association functional class (NYHA), and the six-minute walk test (6-MWT). All follow-up data (median 31 months) were compared with baseline and divided into: short-term (<12 months), mid-term (between 12 and 24 months), and long-term follow-up (>24 months).Results. At baseline, 15 patients were in NYHA class III and three in NYHA class IV, mean log NT-pro BNP level was 7.2+/-1.4 log pg/ml, and mean 6-MWT distance was 404+/-125 m. During short-term follow-up (n=18), the NYHA class improved (p=0.001), 6-MWT distance increased by 33 m (p=0.03), and log NT-pro BNP decreased to 6.9+/-1.4 log pg/ml (p=0.007). During mid-term follow-up (n=17), the NYHA class improved (p<0.001), the mean 6-MWT distance increased by 41 m (p=0.01), and log NT-pro BNP was 6.9+/-1.4 log pg/ml (p=0.31). During late followup (n=14) the NYHA class was still improved (p=0.03), the 6-MWT distance decreased by 9 m (p=0.73), and log NT-pro BNP was 7.1+/-1.5 log pg/ml (p=0.91). The overall four year survival rate was 88%. CONCLUSION: Bosentan seems to be effective during long-term treatment in patients with inoperable CTEPH. (Neth Heart J 2009;17:329-33.).

Three-dimensional transoesophageal echocardiography in a patient undergoing percutaneous mitral valve repair using the edge-to-edge clip technique.

We report a case of percutaneous mitral valve repair, using the Mitraclip device, in which we show that application of real-time three-dimensional transoesophageal echocardiography (3D-TEE) is extremely helpful for the guidance of this procedure. Because of its excellent visualization capacities, 3D-TEE simplifies the transseptal puncture, the positioning of the clip above the mitral valve orifice, the grasping of the mitral valve leaflets, and the evaluation of the final result. Therefore, we conclude that 3D-TEE has the potential to increase the safety and efficacy of this new technique to treat mitral regurgitation in patients who cannot undergo conventional valve surgery.

Mannose binding lectin deficiency and triglyceride-rich lipoprotein metabolism in normolipidemic subjects.

Mannose binding lectin (MBL) is one of the three initiators of complement activation and is therefore closely linked to inflammation. MBL deficiency has been associated with the generation of atherosclerosis. Since atherosclerosis, the complement system and postprandial lipemia are linked to inflammation, we studied postprandial lipoprotein metabolism in MBL deficiency. An observational study was carried out in 107 volunteers (21% MBL deficient). Classical cardiovascular risk factors were not different between subjects with and without MBL deficiency. Oral fat loading tests in 8 MBL deficient and 14 MBL sufficient subjects showed similar postprandial triglyceride, free fatty acid, hydroxybutyric acid and complement component 3 concentrations. MBL deficient subjects had 2.4 times lower postprandial Sf>400 (chylomicron)-apoB48 concentrations, but in contrast a 2-3.5 times increased Sf 60-400 (VLDL1-TG) and Sf 60-400-apoB100 response. MBL activity was inversely related to the postprandial Sf 60-400-TG increase. Despite lower postprandial Sf>400-apoB48 concentrations, MBL deficient subjects show an accumulation of Sf 60-400 lipoproteins.

Pulmonary arteriovenous malformations associated with migraine with aura.

Migraine with aura (MA) is associated with cardiac right-to-left shunt. We prospectively studied the association between pulmonary arteriovenous malformations (PAVMs) and MA in hereditary haemorrhagic telangiectasia (HHT). All 220 consecutive HHT patients who underwent high-resolution chest computed tomography for PAVM screening were included prospectively. Prior to screening, a structured validated headache questionnaire was completed by 196 patients (57% female; mean+/-sd age 44.6+/-15.2 yrs). Two neurologists diagnosed migraine according to the International Headache Society Criteria. A PAVM was present in 70 (36%) patients. The prevalence of MA was 24% in the presence of a PAVM compared with 6% in the absence of a PAVM (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and MA was an independent predictor for the presence of PAVM using multivariate analysis (OR 3.6, 95% CI 1.21-10.5; p = 0.02). A PAVM was present in 68% of the patients with MA compared with 32% in the non-migraine controls (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and a PAVM was an independent predictor for MA using multivariate analysis (OR 3.0, 95% CI 1.00-9.20; p = 0.05). In conclusion, PAVMs are associated with MA in HHT patients.

Long-term outcome after balloon angioplasty of coarctation of the aorta in adolescents and adults: Is aneurysm formation an issue?

OBJECTIVES: The purpose was to evaluate long-term outcome following balloon angioplasty for coarctation in adults. BACKGROUND: Long-term results of balloon angioplasty for native coarctation in adults remain incomplete, especially concerning the occurrence of aneurysm formation. METHODS: Long-term follow-up data were collected in consecutive patients retrospectively. Results of balloon angioplasty (29 patients, age 15-71 years, during 1995-2005) for discrete, native coarctation were evaluated, including MRI or CT. RESULTS: Mean follow-up ranged from 2.2 to 13 years (mean 8.5 +/- 3.2). Immediate success was obtained in all patients. Early mortality or complications were not encountered. Peak systolic pressure gradient decreased from 52 +/- 21 to 7.2 +/- 7.6 mm Hg (P < 0.001). Intima tear was detected in eight procedures angiographically, without signs of dissection. Three-month follow-up angiography in these patients showed unchanged (4/8 patients) or diminished abnormalities (4/8 patients). One asymptomatic patient, known with left ventricular dysfunction due to significant aortic valve insufficiency, died suddenly 5 years after balloon angioplasty. Recoarctation occurred in one patient (3%). Late aneurysm formation was excluded by MR in 24/29 and CT in remaining 5/29 patients during follow-up, including those patients in whom intima tear was encountered immediately postangioplasty. In three of seven patients an irregular aortic contour persisted, without indication of progression or aneurysm formation. Hypertension was completely relieved in 67% (14/21 patients) and improved in 33% (7/21 patients). CONCLUSIONS: Balloon angioplasty for native coarctation yields low reintervention probability in adult patients. Despite occurrence of angiographically established intimal tearing, aortic dissection and aneurysm formation were not encountered.

Comparison of different methods to investigate postprandial lipaemia.

Postprandial hyperlipidaemia has been associated with coronary artery disease (CAD). We investigated which of the generally used methods to test postprandial lipaemia differentiated best between patients with premature CAD (50+/-4 years, n=20) and healthy controls. Furthermore, the effects of rosuvastatin 40 mg/day on postprandial parameters were assessed. Standardised oral fat-loading tests (OFLT) and ambulant self-measurements of daylong capillary triglycerides (TGc) were performed. Total responses of individual lipoproteins, plasma TG (TGp) and remnant-like particle cholesterol (RLP-C) were estimated as area under the curve (AUC). Most AUCs were highest in untreated patients and reached control levels after rosuvastatin. From all AUCs, RLP-C-AUC was best associated to TGp-AUC in untreated patients and controls (adjusted R2=0.84, beta=0.92, p.

Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study.

Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of the present study was to prospectively establish the diagnostic value of transthoracic contrast echocardiography (TTCE) as a screening technique for PAVM using chest high-resolution computed tomography (HRCT) as the gold standard for PAVMs. All consecutive adult patients referred for HHT screening underwent a chest HRCT (n = 299), TTCE (n = 281), arterial blood gas analysis (n = 291), shunt fraction measurement (n = 111) and chest radiography (n = 296). TTCE was positive in 87 (58.8%), 12 (16.7%) and four (6.7%) patients, and chest HRCT was positive in 54 (36.5%), three (4.2%) and zero (0%) patients with a definite, possible and negative clinical diagnosis of HHT, respectively. Two patients with a negative TTCE were diagnosed with PAVMs after computed tomography; in both cases the PAVMs were too small to be treated by embolotherapy. The sensitivity of TTCE was 97% (95% confidence interval (CI) 93.6-98.3) and negative predictive value 99% (95% CI 96.9-99.8). The other diagnostic tests showed a considerable lower diagnostic value. The present prospective study shows that transthoracic contrast echocardiography has an excellent diagnostic value and can be used as an initial screening procedure for pulmonary arteriovenous malformations. The high false-positive rate of transthoracic contrast echocardiography possibly represents microscopic pulmonary arteriovenous malformations.

Complications and mid-term outcome after percutaneous patent foramen ovale closure in patients with cryptogenic stroke.

BACKGROUND: Percutaneous patent foramen ovale (PFO) closure seems to reduce the risk of recurrent thromboembolism. We report the safety and efficacy of percutaneous PFO closure in our centre. METHODS: All patients, >16 years of age, who underwent a percutaneous PFO closure in our centre were included. Reoccurrence of stroke, transient ischaemic attack (TIA) and peripheral thromboembolism were assessed. Periprocedural and midterm complications are reported. RESULTS: Eighty-three consecutive patients (mean age 49+/-13 years) were included. Indications for PFO closure were cryptogenic stroke (59.0%), TIA (33.7%), peripheral embolism (2.4%) and other (4.8%). For PFO closure, a Cardioseal/Starflex device was used in 63 patients and an Amplatzer PFO occluder device in 20 patients. Stroke recurred in 1.2%, TIA in 3.6%, peripheral embolism in 0% during a mean follow-up of 1.9+/-1.2 years. Major periprocedural complications occurred in 1.2%. The mid-term complication rate was 2.4% and only consisted of minor complications. During follow-up, a residual right-to-left shunt was present in 5.7% of the patients. No significant difference in outcome, complications or residual shunting could be documented between the two device types. CONCLUSION: In our centre, the percutaneous closure of a PFO seems to be a safe and effective procedure to prevent recurrence of paradoxical thrombo-embolic events. (Neth Heart J 2008;16:332-6.).

[Unexplained right-sided heart failure following open heart surgery: mind the pericardium]. / Onverklaarde rechtsdecompensatie na een openhartoperatie: denk aan het hartzakje.

Three men, aged 67 years, 80 years and 53 years, respectively, developed signs and symptoms of progressive right-sided heart failure following open heart surgery. They were diagnosed with constrictive pericarditis based on echocardiography, cardiac magnetic resonance and cardiac catheterisation. Following pericardiectomy, two of the patients fully recovered, while one, the 80-year-old man, died during convalescence. When signs and symptoms of progressive right-sided heart failure develop after open heart surgery, a diagnosis of constrictive pericarditis should be considered. Constrictive pericarditis after open heart surgery may be caused by inflammation of the pericardium; an old, fibrotic haemopericardium, which may be diffuse or loculated; pericardial adhesions; or a combination of these entities. Diagnosing constrictive pericarditis is difficult and may take a long time. However, it is important to recognise this disorder early before it has progressed to an advanced stage. Pericardiectomy is the only effective therapy. When performed too late, survival is significantly reduced.

Pulmonary arteriovenous malformations and migraine: a new vision.

Migraine is a common neurological disorder with a great impact on the quality of life and social activities. Pulmonary arteriovenous malformations (PAVMs) are mostly congenital, with a prevalence of 5-50% in patients with hereditary hemorrhagic telangiectasia (HHT). A high prevalence of PAVMs is found in patients with HHT and migraine. Embolization of PAVMs seems to decrease the prevalence of migraine. Different pathophysiological hypotheses have been proposed to explain the association between migraine and the different right-to-left shunts. This review article describes the association between a pulmonary right-to-left shunt and the occurrence of migraine.