Andrzej Biederman

Pulmonary Artery Dilatation Correlates With the Risk of Unexpected Death in Chronic Arterial or Thromboembolic Pulmonary Hypertension

BACKGROUND Right ventricular failure does not explain all cases of death in patients with chronic pulmonary hypertension. Searching for alternative explanations, we evaluated the prognostic significance of main pulmonary artery (PA) dilatation in patients with pulmonary arterial hypertension (PAH) or chronic thromboembolic pulmonary hypertension (CTEPH). METHODS A retrospective outcome analysis was made of 264 patients (aged 46 ± 17 years; women, 69%; PAH, 82%) who underwent both CT scan measurement of the PA and right-sided heart catheterization (mean PA pressure, 57.6 ± 16.5 mm Hg) at initial evaluation. RESULTS The diameter of the PA ranged from 28 to 120 mm (mean, 39 ± 8.6 mm; median, 38 mm) and was largest in patients with unrepaired congenital defects (42.6 ± 7.6 mm). Pulmonary pulse pressure (P = .04), lower age (P = .03), and duration of symptoms (P < .001) were independently but weakly related to PA diameter. During follow-up (median, 38 months), 99 patients (37%) died. Of these 99 deaths, 73 (74%) were due to heart failure or comorbidities, and 26 (26%) were unexpected deaths (UE-Ds). PA diameter (hazard ratio [HR], 1.06 per 1 mm; 95% CI, 1.03-1.08), heart rate (HR, 1.30 per 10 beats/min; 95% CI, 1.01-1.66), and systolic pulmonary arterial pressure (HR, 1.02 per 1 mm Hg; 95% CI, 1.01-1.04) were the only independent predictors of UE-D and differed from the usual predictors found in the study group for all-cause mortality. PA diameter ≥ 48 mm had 95% specificity and 39% sensitivity and carried 7.5 times higher risk of UE-D (95% CI, 3.4-16.5; P < .0001) during follow-up. CONCLUSIONS PA dilatation emerges as an independent risk factor for death unexplained by right ventricular failure or comorbidities in patients with PAH and CTEPH. The possible mechanisms include, but are not limited to, PA compression of the left main coronary artery, PA rupture, or dissection with cardiac tamponade.

Outcome of Medically Versus Surgically Treated Patients With Chronic Thromboembolic Pulmonary Hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an ominous disease leading to progressive right heart failure. Selected patients can be treated by pulmonary endarterectomy (PEA). We assessed long-term clinical outcome of patients with CTEPH who underwent PEA and patients who remained on medical treatment alone. A total of 112 consecutive patients with CTEPH referred between 1998 and 2008 to one center were followed for a mean of 35 (range 0-128) months after diagnosis. All the patients had advanced pulmonary hypertension at baseline. The operated group had higher World Health Organization functional class compared to the nonoperated group. No other differences in hemodynamic, echocardiographic, or biochemical parameters were observed at baseline. Despite the perioperative mortality rate of 9.1%, patients who underwent PEA had significantly lower long-term mortality compared to nonoperated patients (12.7% vs 34.8%; P = .003), and PEA survivors showed sustained clinical improvement. All efforts should be undertaken to perform PEA in all patients with operable CTEPH.

Improvement in Quality of Life and Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension Treated With Balloon Pulmonary Angioplasty

BACKGROUND The effect of balloon pulmonary angioplasty (BPA) on improvement in functional and hemodynamic parameters in chronic thromboembolic pulmonary hypertension (CTEPH) is known, but the quality of life (QoL) of patients treated with BPA has never been studied before.Methods and Results:Twenty-five patients with inoperable or persistent CTEPH were enrolled in the study and filled out the 36-item Short Form (SF-36v2) questionnaire twice: prior to commencement of BPA treatment and after ≥3 BPA sessions. In addition WHO functional class, distance on the 6-min walk test (6MWT) and hemodynamic parameters such as right atrial pressure (RAP), mean pulmonary artery pressure (mPAP), cardiac index (CI) and pulmonary vascular resistance (PVR) were assessed. QoL improved significantly in all domains, except for physical pain. Improvement in RAP (10.5±3.4 vs. 6.2±2.2 mmHg; P<0.05), mPAP (51.7±10.6 vs. 35.0±9.1 mmHg; P<0.05), CI (2.2±0.5 vs. 2.5±0.4 L/min·m2; P=0.04), PVR (10.4±3.9 vs. 5.5±2.2 Wood units; P<0.05), functional class (96% vs. 20% in WHO class III and IV, P<0.05) and improvement in 6MWT distance (323±135 vs. 410±109 m; P<0.05) was observed. The only significant correlation was between the mental component summary score of QoL after completion of treatment and percentage improvement in the 6MWT (-0.404, P<0.05). CONCLUSIONS Alongside improvement in functional and hemodynamic parameters, BPA also provides significant improvement in QoL.

Transapical off-pump mitral valve repair. First experience with the NeoChord system in Poland (report of two cases)

BACKGROUND Artificial chord implantation to repair a flail or prolapsing mitral valve leaflet requires open heart surgery and cardiopulmonary bypass. AIM Transapical off-pump artificial chordae implantation is a new surgical technique proposed to treat degenerative mitral valve regurgitation. The procedure is performed using the NeoChord DS1000 system (NeoChord, Inc., St. Louis Park, MN, USA), which facilitates both implantation and lenght adjustment of the artificial chordae under two (2D)- and three (3D)-dimensional transoesophageal echocardiographic (TEE) guidance on a beating heart. METHODS Two male patients aged 60 and 55 years with severe mitral regurgitation due to posterior leaflet prolapse underwent transapical off-pump artificial chordae implantation on September 3, 2015. The procedure was performed by left minithoracotomy under general anaesthesia in a cardiac surgical theatre, using 2D and 3D TEE guidance. RESULTS Early procedural success as confirmed by 3D TEE was achieved in both patients, with implantation of 6 artificial chordae in the first patient and 3 artificial chordae in the second patient. Both procedures were uneventful, and no postoperative complications were noted. The patients were discharged home on the 8th and 6th postoperative day, respectively. CONCLUSIONS The NeoChord DS1000 system allows both implantation and lenght adjustment of artificial chordae under 2D and 3D TEE guidance on a beating heart. Our initial experience in 2 patients with posterior mitral leaflet prolapse indicates that the procedure is feasible and safe.

Acute and chronic dissection of pulmonary artery: new challenges in pulmonary arterial hypertension?

Right ventricular failure is a leading cause of mortality in patients with pulmonary arterial hypertension (PAH). However, up to 25% of such patients die unexpectedly, without warning signs of hemodynamical decompensation. We previously documented that pulmonary artery (PA) dilatation significantly increases the risk of those deaths. Some of them may be due to dissection of PA resulting in cardiac tamponade. However, direct confirmation of this mechanism is difficult as most of such deaths occur outside hospitals. We present 4 patients with severe PAH and PA dilatation in whom PA dissection has been confirmed. Three patients had IPAH, one had PAH associated with congenital heart disease. All patients had mean pulmonary artery pressure (PAP) > 50 mmHg at diagnosis and dissection occurred late in the course of apparently well controlled disease (6 to 14 years). Several clinical elements were common to our patients - high systolic PAP, long lasting PH, progressive dilatation of PA to more than 50 mm with chest pain prior to dissection. However, clinical course followed three different patterns: sudden death due to cardiac tamponade, hemopericarditis caused by blood leaking from dissected aneurysm with imminent but not immediate cardiac tamponade, or chronic asymptomatic PA dissection. Indeed, two of our patients are alive and on lung transplantation waiting list for more than 2 years now. Further research is needed to suggest optimal management strategies for patients with stable PAH but significantly dilated proximal pulmonary arteries or confirmed PA dissection depending on the clinical presentation and expected outcome.

Balloon pulmonary angioplasty for the treatment of residual or recurrent pulmonary hypertension after pulmonary endarterectomy

BACKGROUND Pulmonary endarterectomy (PEA) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). However, persistent pulmonary hypertension continues in 5-35% of patients after PEA. Recently, balloon pulmonary angioplasty (BPA) showed promise as a strategy for patients with non-operable CTEPH. Therefore, we investigated the usefulness of BPA for residual pulmonary hypertension after PEA. METHODS Fifteen patients with residual pulmonary hypertension after PEA received 71 BPA sessions (4.7 ± 1.4 sessions/patient). The mean time between the PEA and the first BPA session was 28.1 ± 25.8 months. All patients underwent a comprehensive diagnostic work-up, including right heart catheterization, functional and laboratory tests, before, and 6-4 weeks after the BPA sessions. RESULTS After BPA, the mean pulmonary arterial pressure decreased from 44.7 ± 6.4 to 30.8 ± 7.5 mm Hg (31% decline; p < 0.001). Pulmonary vascular resistance decreased from 551.9 ± 185.2 to 343.8 ± 123.8 dyn∗s/cm-5 (38% decline; p < 0.001). The 6-min walking distance increased from 383 ± 104 to 476 ± 107 m (mean change +93 m; p < 0.001). In two sessions (2.8%), serious periprocedural complications occurred. During a mean follow-up of 18 ± 14.3 months, one patient died two months after the last BPA session. Fourteen patients survived. CONCLUSIONS BPA could be a promising therapeutic strategy for persistent pulmonary hypertension after PEA in patients with CTEPH.

A statement the Polish Cardiac Society Working Group on Pulmonary Circulation on screening for CTEPH patients after acute pulmonary embolism

Both pharmacological and invasive treatment of chronic thromboembolic pulmonary hypertension (CTEPH) is now available in Poland and the awareness of the disease among physicians is growing. Thus, the Polish Cardiac Societys Working Group on Pulmonary Circulation in cooperation with independent experts in this field, have launched the statement on algorithm to guide a CTEPH diagnosis in patients with previous acute pulmonary embolism (APE). In Poland, every year this disease affects about 250 patients. CTEPH should be suspected in individuals after APE with dyspnea, despite at least 3 months period of effective anticoagulation, particularly when specified risk factors are present. Echocardiography is a main screening tool. The authors suggest that a diagnostic process of patients with significant clinical suspicion of CTEPH and right ventricle overload in echocardiography should be performed in reference centres. The document contains a list of Polish centres diagnosing patients with suspected CTEPH. Pulmonary scintigraphy is a safe and highly sensitive screening test for CTEPH. Multi-detector computed tomography with precise detection of thromboembolic residues in pulmonary circulation is important for planning of pulmonary endarterectomy. Right heart catheterisation definitely confirms the presence of pulmonary hypertension and direct pulmonary angiography allows for identification of lesions suitable for thromboendarterectomy or pulmonary balloon angioplasty. In this document a diagnostic algorithm in patients with suspected CTEPH is also proposed. With individualised sequential diagnostic strategy each patient can be finally qualified for a particular mode of therapy by dedicated CTEPH Heart Team. Moreover the document contains short information for the primary care physician about the management of patients after APE.

Isolated mitral valve P3 prolapse with severe regurgitation – transoesophageal 2D/3D echo-guidance in transapical -artificial neochordae implantation in challenging patient

Received 12 April 2016; revision accepted for publication 30 May 2016. A 60-year-old man without significant medical history was admitted due to a loud heart murmur and increasing exercise intolerance. Transthoracic (TTE) and transoesophageal (TEE) 2D, and real-time 3D echocardiography revealed a large coaptation defect of mitral valve (MV) leaflets caused by P3 prolapse with chordal rupture (figure 1 A, D-arrows). We diagnosed severe mitral regurgitation with a wide eccentric jet in colour Doppler (figure 1B), vena contracta width approximately 7 mm, volume > 60 ml, systolic pulmonary vein flow reversal and left atrium enlargement. There was no significant annular dilatation (syst. 37 mm/diast. 39 mm and mitral annulus/anterior leaflet ratio approximately 1.3 in LAX). Coronary angiography showed no significant lesions in the arteries. The patient was qualified for an innovative minimally invasive surgical procedure of transapical beating-heart implantation of polytetrafluoroethylene neochordae with the NeoChord DS1000 system, through a mini-thoracotomy without cardiopulmonary bypass. Under TEE 2D/3D guidance the Isolated mitral valve P3 prolapse with severe regurgitation – transoesophageal 2D/3D echo-guidance in transapical artificial neochordae implantation in challenging patient

Right atrial myxoma with pulmonary embolism

We present a rare case of a patient with giant right atrial myxoma and simultaneous pulmonary embolism, with good results after surgical removal of the tumour. A 62-year-old woman was admitted to the hospital due to fatigue increasing for several weeks before admission. She complained of progressive exertional dyspnoea and had a medical history of three-months anaemia. On admission she was in a good general condition without clinical signs of heart failure. Transthoracic and transoesophageal echocardiography revealed a mobile mass about 7 cm in length attached to the posterior wall of the right atrium (Figs. 1–3). It prolapsed through the tricuspid valve into the right ventricular outflow tract during diastole. Examinations did not reveal any pathological structures in the main veins draining into the right atrium. Computer tomography (CT) of the chest showed a solid, heterogeneously-hypodense mass, poorly delimited in the right atrium and ventricle, and with dimensions 80 × 45 mm. The lower limb compression venous Doppler ultrasonography showed no signs of thrombosis. Moreover, the pulmonary embolism was confirmed by angio-CT. It revealed the multiple emboli in lobar and segmental pulmonary arteries. The patient was urgently qualified for cardiac surgery and underwent it without complications. The tumour was completely removed (Fig. 4). Histological examination of the tumour confirmed myxoma. Primary tumours of the heart coexisting with a pulmonary embolism are rare. Right atrial myxoma is observed only in 15–20% of myxoma cases with high potential of pulmonary embolism. Immediate surgical treatment is indicated on account of the high risk of pulmonary embolism or sudden cardiac death.

Giant Metastatic Intrapericardial Tumor 20 Years After Nephrectomy

We present a rare case of giant, metastatic renal cell carcinoma of the pericardium diagnosed 20 years after nephrectomy. An endovascular procedure was used to place coils preoperatively in the large collateral vessels supplying the tumor to achieve mass reduction and reduce intraoperative bleeding. The tumor was resected through a median sternotomy, and the patients recovery was uneventful. Preoperative coil placement in the tumoral arteries enabled a safe and bloodless resection in this rare presentation.