Pulmonary Hypertension Associated With Myocardial Amyloid Degeneration: a Case Report

Background: There is very little literature on Pulmonary hypertension associated with myocardial amyloid degeneration. At present, only 10 cases pulmonary hypertension cased by amyloid protein deposits in the pulmonary blood vessels have been reported by Eder et al. We reported a case that the patient was pulmonary artery hypertension combined with myocardial amyloid change. It’s aim to claims that pulmonary hypertension is most likely caused by amyloid brin deposition in pulmonary blood vessels. Case presentation: We report a case of a 65-year-old male patient with with AL and ATTR combined type amyloidosis who developed right heart failure because of severe pulmonary hypertension. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon nding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis. Conclusion: we present a men with amyloidosis who developed dyspnea and right heart failure and was diagnosed with pulmonary hypertension, most probably secondary to pulmonary vascular involvement by amyloid brils.

amyloidosis who developed right heart failure because of severe pulmonary hypertension. Pulmonary hypertension due to deposition of amyloid in the pulmonary vasculature is an uncommon nding; however, it should be considered in cases of unexplained pulmonary hypertension in patients with amyloidosis.
Conclusion: we present a men with amyloidosis who developed dyspnea and right heart failure and was diagnosed with pulmonary hypertension, most probably secondary to pulmonary vascular involvement by amyloid brils.

Case Presentation
Male, 65 years old. Shortness of breath 4 years after activities, worsened for 3 months. Since 2016 patients began to appear shortness of breath, no other discomfort. Echocardiogram: left atrium (LA) : 36mm, Left ventricular end diastolic dimension (LVEDD) : 46mm, right ventricular (RV) : 38mm, estimated pulmonary artery systolic pressure (sPAP) 132mmHg; pulmonary artery CTA: no section and above embolism; pulmonary ECT: no clear pulmonary embolism lesions image. Right heart catheter examination: right arrhythm average pressure 4mmHg, pulmonary artery pressure 77/26/45mmHg, pulmonary small artery wedge pressure 4mmHg, acute vascular reaction test: negative. Pulmonary angiography : a narrow or closed part of the pulmonary artery is visible. Consider that the patient is chronic thromboembolism pulmonary hypertension (CTEPH), treated with Bosantan, Warfarin and other drugs. The patient feeled that the symptoms are getting worsen 2020, so in hospital again, and treated by balloon pulmonary angioplasty and drugs, the improvement was not obvious. Dec 2020, the patient hospitalized in Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences for further treatment.
The patient smoking for 30 years,had given up 4 years, had no other special medical history and denied high blood pressure or diabetes.
The patient was treated with low-molecular anticoagulant, diuretic, Levosimendan injection strong heart, gastric care, Bosantan targeted drug .
Right heart catheter examination (Table 1): cavity vein oxygen saturation 64.25, right agency oxygen saturation 62.67, right heart chamber oxygen saturation 60.15, pulmonary artery oxygen saturation 59.7, arterial oxygen saturation 59.7; 14mmHg, right culmonary pressure 78/0/11mmHg, pulmonary artery pressure 79/36/50mmHg, pulmonary small artery wedge pressure 12/13/12mmHg; QP/QS 0.87, CI 2.44L/min/m2, pulmonary vascular resistance 11.38 Wood. Pulmonary artery atomtomy (Fig. 2): aortic artery widening, left and right pulmonary artery near-end dilation, each segment, sub-section of the pulmonary artery did not see stenosis and lling defects, tube cavity smooth, slow blood ow, far-end perfusion is not uniform, the corresponding venous re ow shallow. Cardiocardial endometrial biopsy: myocardial amyloid degeneration, compound type. Immune histi cation results: TTR (-), APO A1 (-), APO AII (-), APO AIV (-), AA (-), Kappa (-), Lambda (?). Electroscope: a small number of cardiomyocyte collagen ber dissolved, did not see collagen ber cross-arrangement. The number of mitochondrials is slightly larger, mitochondrial swelling and dissolution is rare, there are not many lipid droplets and lipolytin in cells, no glycogen accumulation, no myelin-like structure. The substrate of myocardial cells was clear, no T-tube expansion was seen, and no abnormality was seen in the structure of the leap disk. The intermyocardial mass is found in a large number of branchless ber structures, which are deposited with amyloid.

Discussion
Amyloidosis is the general term used to refer to the extracellular tissue deposition of brils composed of low molecular weight subunits of a variety of proteins, many of which circulate as constituents of plasma. These deposits may result in a wide range of clinical manifestations depending upon their type, location, and the amount of deposition [1]. The clinical manifestations of amyloidosis are diverse, depending on the pattern of organ involvement. The variable clinical phenotype and generally nonspeci c clinical features makes diagnosis di cult and contributes to diagnostic delays.Age of onset and disease distribution -The usual age of onset of symptoms and disease distribution varies among the various types of amyloidosis [2]. Pulmonary hypertension (PH) hemodynamically de ned as mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg by right heart catheterization (RHC) at rest is a progressive and fatal condition which usually culminates in right heart failure and death [3].
At present, only 10 cases pulmonary hypertension cased by amyloid protein deposits in the pulmonary blood vessels have been reported by Eder et al [4]. We describe an exception to the hospital rst diagnosis of CTEPH, by targeted drug treatment and balloon pulmonary angioplasty (BPA), the effect is not good.
Although the patient has evidence of heart amyloid protein location, but in previous reports, amyloid tiredness and myocardial muscle often cause myocardial throesopathy function is limited, will not cause serious pulmonary hypertension. The most frequent cardiac presentation is that of a restrictive cardiomyopathy with diastolic dysfunction. Right-sided heart failure secondary to PH from vascular amyloid deposits is rare [5][6][7][8].
After perfect examination, we can make it clear that the patient is the rst class of pulmonary hypertension, but the patient through targeted drug treatment improvement is not obvious. Combined with evidence of amyloid change, we have more reason to suspect that the patient was caused by amyloid protein deposits in the pulmonary arteries.
The mechanism of myocardial amyloid change and pulmonary hypertension: the deposition of light chains appears to have tissue speci city that may depend on the molecular characteristics of the amyloid protein [9]. It has been reported that amyloid deposition causes endothelial dysfunction and proliferation of vascular smooth muscle cells [10].
In summary, it can be clear that the patient was pulmonary artery hypertension combined with myocardial amyloid change. Pulmonary hypertension is most likely caused by amyloid brin deposition in pulmonary blood vessels.

Declarations
Ethics approval and consent to participate: The study was performed with the approval of