Clinical relevance of adding intravascular ultrasound to coronary angiography for the diagnosis of extrinsic left main coronary artery compression by a pulmonary artery aneurysm in pulmonary hypertension.

OBJECTIVES: We sought to assess the clinical value of adding intravascular ultrasound (IVUS) evaluation to coronary angiography (CA) to guide extrinsic left main coronary artery (LMCA) compression diagnosis and treatment in pulmonary hypertension (PH). BACKGROUND: LMCA compression due to a pulmonary artery aneurysm (PAA) is a severe complication of PH. Although guidelines encourage the use of IVUS for LMCA disease evaluation, it has hardly been used in this scenario. METHODS: We analyzed morbimortality of type 1 and 4 PH patients with clinically suspected LMCA compression by a PAA between 2010 and 2018 in a reference unit. LMCA compression was prospectively assessed with CA ± IVUS. Angiographic-LMCA compression was considered conclusive when LMCA stenosis>50% was present in four predetermined projections; inconclusive, when LMCA stenosis>50% was present in <4 projections and negative if no stenosis>50% was present. Patients with conclusive and inconclusive CA underwent IVUS. IVUS-LMCA compression was defined as systolic minimum lumen area < 6 mm2 . RESULTS: LMCA compression was suspected in 23/796 patients (3%). CA was conclusive for compression in 7(30.5%), inconclusive in 9(39%), and negative in 7(30.5%). IVUS confirmed LMCA compression in 6/7(86%) patients with conclusive CA and in 2/9(22%) with inconclusive CA. Patients fulfilling IVUS criteria for LMCA compression underwent stent implantation. At 20 months follow-up a composite end-point of death, stent restenosis/thrombosis, or lung transplant was reported in three patients (13%). CONCLUSIONS: CA can misdiagnose LMCA extrinsic compression. IVUS discriminates better whether significant compression by a PAA exists or not, avoiding unnecessary LMCA stenting. Patients treated following this strategy show a low rate of major clinical events at 20 months follow-up.

A rare childhood case of Behcet's disease and chronic thromboembolic pulmonary hypertension.

BACKGROUND: Pulmonary embolism and chronic thromboembolic pulmonary hypertension (CTEPH) are rare complications of Behcet's disease, especially in pediatric patients. AIMS/METHODS/RESULTS/CONCLUSIONS: This case report highlights a presentation of CTEPH in an adolescent with Behcet's disease. A multidisciplinary approach was required for managing this patient's CTEPH, which successfully reversed the patient's pulmonary hypertension.

Comparative analysis of pulmonary hypertension in patients treated with imatinib, nilotinib and dasatinib.

Pulmonary hypertension (PH) is a rare, but life-threatening, adverse event in patients treated with tyrosine kinase inhibitors (TKIs), such as dasatinib, but has not been fully evaluated in patients treated with imatinib or nilotinib. We used echocardiography to noninvasively assess the incidence of PH in 105 patients with chronic myeloid leukaemia (CML) treated with imatinib (n = 37), nilotinib (n = 30) or dasatinib (n = 38). The mean triscupid regurgitation peak gradient (TRPG), which reflects pulmonary arterial pressure, was 22·7 mmHg in the imatinib group, 23·1 mmHg in the nilotinib group and 23·4 mmHg for dasatinib group. These values were not significantly different, but higher than those (19·0 mmHg) in newly diagnosed CML patients. A TRPG > 31 mmHg, marking possible PH onset, was detected in 9 of 105 patients: one (2·7%) treated with imatinib, three (10·0%) with nilotinib and five (13·2%) with dasatinib. Only three patients complained of dyspnoea, whereas the other six were asymptomatic. In addition, there was a tendency toward correlation of TRPG value and age or TKI treatment duration. These results suggested that treatment with not only dasatinib, but also imatinib and nilotinib, can be associated with subclinical PH. Noninvasive echocardiography is useful for screening, especially in older patients with long-term TKI treatment.

Comparison of the therapeutic effects and side effects of tadalafil and sildenafil after surgery in young infants with pulmonary arterial hypertension due to systemic-to-pulmonary shunts.

Young children with CHD and large systemic-to-pulmonary shunts eventually develop pulmonary hypertension. At present, phosphodiesterase type-5 inhibitors such as sildenafil have been used to control pulmonary pressure before and after cardiac surgery. Recently, tadalafil has been utilised in older children with similar efficacy, but it has been used to a lesser extent in young infants. From April, 2015 to June, 2016, 42 patients aged 3-24 months with a large septal defect and pulmonary arterial hypertension were randomly divided into two equal groups: one group received oral sildenafil (1-3 mg/kg/day every 8 hours), whereas the other group received oral tadalafil (1 mg/kg once a day) from 7-10 days before surgery to 3-4 weeks after surgery. During the first 48 hours after surgery, pulmonary artery-to-aortic pressure ratio and recorded systolic pulmonary artery pressures were not significantly different between the two groups (p>0.05); moreover, there were no differences in paediatric ICU length of stay, mechanical ventilation time, clinical findings of low cardiac output state, and echocardiographic data between the two groups (p>0.05). Most of the patients had no side effects, and only five patients had a minor with no significant difference in both groups (p=0.371). Tadalafil can be considered as an effective oral therapy for preoperative and postoperative pulmonary hypertension in young infants. It can be administered at a once-daily dose with an appropriate efficacy and safety profile as sildenafil, and therefore it can be considered as an alternative to sildenafil in young children.

The evolving role of cardiac magnetic resonance imaging in the assessment of cardiovascular disease.

BACKGROUND: Imaging of the heart is important in the diagnosis and follow-up of a broad range of cardiac pathology. The authors discuss the growing role of cardiac magnetic resonance imaging (CMR) in cardiology practice and its relevance to primary healthcare. OBJECTIVE: In this article we discuss the advantages of CMR over other imaging modalities, and give a brief description of the common CMR techniques and cardiac pathologies where CMR is especially useful. DISCUSSION: CMR provides specific advantages over other cardiac imaging modalities when evaluating pathology in congenital heart disease, cardiac masses, cardiomyopathies, and in some aspects of ischaemic and valvular heart diseases. The strength of CMR in these pathologies includes its precise ana-tomical delineation of structures, characterisation of myocardial tissue, and accurate, reproducible measurements of blood volume and flow. CMR is used in inpatient and outpatient settings, and is available primarily in major hospitals.

Cardiac function and its evolution with pulmonary vasodilator therapy: a myocardial deformation study.

Speckle tracking echocardiography-derived myocardial strain has useful clinical applications in adults with pulmonary hypertension (PH) as well as preterm infants with chronic lung disease. It is considered more sensitive compared to conventional indices. This report presents a 3-month-old infant with PH and poor right ventricular function who was treated with inhaled nitric oxide. Myocardial strain was noted to be impaired with paradoxical segmental strain. Impairment in strain improved after inhaled nitric therapy. Strain analysis can help improve understanding of cardiac adaptation in critical clinical situations.

Do Right Heart Hemodynamic Improvements Persist After Pulmonary Thromboendarterectomy?

The survival benefits of pulmonary thromboendarterectomy (PTE) for the treatment of chronic thromboembolic pulmonary hypertension have been well described. However, the significance of right heart hemodynamic changes and their impact on survival remains poorly understood. We sought to characterize the effects of these changes. We conducted a single center, retrospective review of 159 patients who underwent PTE between 1993 and 2015. Echocardiographic and right heart catheterization data were compared longitudinally before and after PTE in order to establish the extent of hemodynamic response to surgery. Kaplan Meier estimates were used to characterize patient survival over time. Univariable and multivariable Cox proportional hazards regression models were used to assess factors associated with long-term mortality. Among the 159 patients studied, 74 (46.5%) were male with a median age of 55 (IQR: 42-66). One-, 5-, 10-, and 15-year survival was 91.0% (95% CI: 86.6-95.6), 79.6% (73.5-86.3), 66.5% (59.2-74.7), and 56.2% (48.1-65.8). Of the 9 candidate risk factors that were evaluated, only advanced age and increased cardiopulmonary bypass time were found to be significantly associated with increased risk of mortality. Pre- and postsurgical echocardiographic imaging data, when available, revealed a median reduction in right ventricular systolic pressure of 29.0 mm Hg (P < 0.0001) and improvement of tricuspid regurgitation (P < 0.0001), both of which appeared to be sustained across long-term follow-up. Improvements in right heart hemodynamics and tricuspid valvular regurgitation persist on long term surveillance following PTE. While patient selection is often driven by the distribution of disease, close postoperative follow up may improve outcomes.

Isolated congenital left-sided pulmonary vein stenosis in an adult patient misdiagnosed as primary pulmonary hypertension.

Pulmonary vein stenosis (PVS) is a rare condition, seen usually in association with congenital heart disease or secondary to various acquired causes. Isolated PVS, in adults, especially in absence of congenital heart disease is extremely uncommon. We report PVS of left sided pulmonary veins in an 18-year-old male, who had been till then diagnosed as primary pulmonary hypertension (PPH).

Long-term remission of pulmonary veno-occlusive disease associated with primary Sjögren's syndrome following immunosuppressive therapy.

The patient described here is a 21-year-old Japanese woman with primary Sjögren's syndrome (pSS) presenting with worsening of dyspnea, palpitation, recurrent parotitis, and arthritis. Chest computed tomography showed diffuse interlobular septal thickening and ground-glass opacities. Right heart catheterization demonstrated pulmonary hypertension, right-sided heart failure, normal pulmonary capillary wedge pressure, and no evidence of arterio-venous shunt. Transbronchial lung biopsy showed luminal obliteration of pulmonary venules by intimal cellular proliferations, without abnormalities in the small pulmonary arteries. These findings were consistent with pulmonary veno-occlusive disease (PVOD). Immunosuppressive therapy, starting with prednisolone 20 mg/day and subsequently combined with azathioprine, resulted in the disappearance of the signs and symptoms, including exertional dyspnea and abnormal pulmonary parenchymal shadows on computed tomography, and the normalization of pulmonary artery pressure. So far, there have been no reported cases of PVOD associated with pSS. Of interest, immunosuppressive therapy without vasodilator therapy almost completely resolved the pulmonary hypertension in this patient.

Reversible Parkinsonism and Multiple Cerebral Infarctions after Pulmonary Endarterectomy in a Patient with Antiphospholipid Syndrome.

Antiphospholipid syndrome (APS) is a cause of chronic thromboembolic pulmonary hypertension (CTEPH) and it is associated with an increased risk of postoperative neurological complications. We experienced a case of reversible parkinsonism after pulmonary endarterectomy (PEA) and subsequent multiple cerebral infarctions under standard anticoagulation therapy in a patient with CTEPH associated with APS. Strict management using a combination of antiplatelet and anticoagulation therapy should be considered in patients with a high titer of triple antiphospholipid antibodies in the perioperative period. We should be aware of the high risk of postoperative neurologic manifestations in patients with APS.

[Diagnosis and treatment of chronic thromboembolic pulmonary hypertension in Denmark].

Chronic thromboembolic pulmonary hypertension (CTEPH) is an important differential diagnosis in patients with unexplained dyspnoea. CTEPH is under-recognized and carries a poor prognosis without treatment. Surgical pulmonary endarterectomy is the preferred treatment for the majority of patients. Advances in surgical and anaesthetic techniques and post-operative intensive treatment have reduced perioperative morbidity and mortality. Pulmonary endarterectomy results in major improvement of haemodynamics and clinical status and offers excellent long-term survival. It is most often a curative treatment. The surgical treatment of CTEPH in Denmark is centralized at Aarhus University Hospital. Pulmonary vasodilators and pulmonary balloon angioplasty are supplementary treatment options in this patient group.