◐ [세균맨 내과 전문의] 순환기 3 ◐ 교착성 심막염, Constrictive pericarditis

◐ [세균맨 내과 전문의] 순환기 3 ◐ 교착성 심막염, Constrictive pericarditis

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▣ [세균맨 내과 KMLE] 순환기 3 ▣ 교착성 심막염, Constrictive pericarditis
에 대한 블로그 내용입니다.  https://blog.naver.com/sjloveu2/221311311116

1. 60대/남자, 1개월 전부터 심해지는 호흡곤란으로 내원하였다. 폐결핵 치료를 받은 적이 있다. 경정맥이 확장되어 있으며 흡기 시에도 확장이 지속되었다. 흉부 엑스선 사진이 다음과 같을 때 진단은?

정답) Constrictive pericarditis
해설) 흡기시 sternal angle에서 경정맥 engorgement의 tip간 수직거리가 6 cm에서 8 cm으로 증가된 것은 Kussmaul's sign입니다. Kussmaul’s sign은 ②constrictive pericarditis 또는 restrictive cardiomyopathy에서 볼 수 있고 EF이 감소된 일부 HF 환자에서 볼 수 있습니다.
  
일반적으로 JVP는 흡기 동안 감소합니다(따라서 jugular venous pulsation의 높이가 흡기 시에 쇄골을 향해서 목 아래 쪽으로 움직입니다). 그러나 일부 환자의 경우 흡기 동안 JVP가 감소하지 않거나 증가합니다. 이 비정상적인 소견을 Kussmaul's sign이라고 합니다. 

! Kussmaul's sign
㉮ Constrictive pericarditis
㉯ RV infarction
㉰ Restrictive cardiomyopathy
※ Cardiac tamponade에서는 Kussmaul's sign이 보통 존재하지 않습니다.

2. Tuberculous pericarditis에 대한 O, X 문제
1) Corticosteroid의 사용이 증상 완화에 도움이 된다. (X)
2) Corticosteroid의 사용이 fluid reaccumulation을 줄인다. (X)
     - Systematic reviews and meta-analyses show that adjunctive glucocorticoid treatment remains controversial, with no conclusive evidence of benefits for principal outcomes of pericarditis - i.e., no significant impact on resolution of effusion, no significant difference in functional status after treatment, and no significant reduction in the frequency of development of constriction or death. However, in HIV-infected patients, glucocorticoids do improve functional status after treatment. [Harrison's 19th edition]

3. 50대/남자, 호흡곤란으로 내원하였다. 20년 전 폐결핵, 결핵성 늑막염을 앓은 병력이 있으며 JVP가 상승되어 있고 간이 2횡지 촉지되었다. 또한 양층 정강이의 함요 부종이 있었다. 진단은?

① 폐동맥협착증
② 유착성 심장막염
③ 삼첨판역류증
④ 승모판협착증
⑤ 승모판역류증

정답 : ② 유착성 심장막염

호흡곤란, 폐결핵/결핵성 늑막염 과거력, Rt side heart failure sign/symptoms(JVP 상승, 간비대 소견, 말단 부종)과 함께 좌측 사진(LV & RV diastolic Pr.)의 diastolic dip and plateau(“square root sign”) pattern과 우측 사진(wedge Pr)의 early systolic and early diastolic dips으로 보아 진단은 constrictive pericarditis입니다.

Cardiac tamponade에서 주어지는 문제의 힌트는 심전도의 electrical alternans, QRS complex의 amplitude 감소, cardiomegaly, JVP 상승, prominent x descent with absent y descent, paradoxical pulse입니다. 반면에 유착성 심장막염에서 주어지는 문제의 힌트는 prominent x descent, y descent 모두 존재입니다.  Prominent x descent는 atrial relaxation 부분이고 우측 사진(wedge Pr)의 early systolic dips입니다. Prominent y descent는 ventricular filling(tricuspid opening) 부분이고 우측 사진(PCW)의 early diastolic dips입니다. Ventricular filling 시작 이후에 ventricle은 갑자기 단단해진 pericardium에 제한되는데(rapid filling wave and plateau) 이것은  square root sign으로 나타납니다.

유착성 심막염 문제 지문에 과거력으로 폐결핵/결핵성 늑막염이 있는 경우가 있습니다. 1962년 한 연구에서는 constrictive pericarditis의 49 %까지 차지하였으나 선진국에서는 이제는 드문 원인 중의 하나입니다. 현재는 HIV 감염 환자와 endemic 지역 환자를 포함하여 활동성 결핵 발생 위험이 높은 환자들에서 감별 원인으로 고려되어야 합니다. 우리나라는 당연히 활동성 결핵 발생 위험이 높은 국가이므로 유착성 심막염 문제에서 폐결핵/결핵성 늑막염 과거력 힌트가 주어지는 것 같습니다.

JVP 상승보다는 덜 일반적이지만 유착성 심막염의 중요한 특징으로는 pulsus paradoxus(20% 미만에서만 존재), Kussmaul's sign, pericardial knock, edema, ascites, cachexia, pulsatile hepatomegaly(part of the syndrome of congestive hepatopathy)가 있습니다.

!
㉮ Constrictive pericarditis
     : Prominent x descent, prominent y descent (RA, LA, PCWP, JVP)
     : Square root sign (RV, LV)
     : Kussmaul's sign (+)
     : Pericardial knock
㉯ Cardiac tamponade
     : Prominent x descent, absent y descent
     : Kussmaul's sign (-)
     : Pulsus paradoxus(가장 흔한 질환은 cardiac tamponade입니다, 
       Constrictive pericarditis에서는 20% 미만만 있습니다)
     : ECG - Electrical alternans, QRS amplitude 감소


4. 70대/남자, 3달 전 CABG를 시행 받았다. 경과 관찰 위해 시행한 2D 심초음파에서 constrictive pericarditis 소견이 보일 때 처치는?

정답) Conservative treatment

해설) [U] Constrictive pericarditis의 원인은 다음과 같습니다. Constrictive pericarditis can occur after virtually any pericardial disease process. The following causes of constrictive pericarditis were identified in case series from tertiary care centers.
· Idiopathic or viral – 42 to 61 percent
· Post-cardiac surgery – 11 to 37 percent
· Post-radiation therapy – 2 to 31 percent, primarily after Hodgkin disease or breast cancer
· Connective tissue disorder – 3 to 7 percent
· Postinfectious (tuberculous or purulent pericarditis) – 3 to 15 percent
· Miscellaneous causes (malignancy, trauma, drug-induced, asbestosis, sarcoidosis, uremic pericarditis) – 1 to 10 percent

CABG 후의 early cardiac complicatoins에서 pericarditis, pericardial effusion, tamponade에 대한 내용입니다. The presentation and clinical course of pericarditis after CABG, which is due to pericardial injury and known as a postpericardiotomy syndrome, is comparable to that of the post-MI syndrome. The most frequent complaint is chest pain, occurring a few days to several weeks after surgery.  

Serial echocardiography shows that postoperative pericardial effusion is considerably more common than clinically apparent disease, occurring in as many as 85 percent of patients. The effusion is usually present by the second postoperative day, but may not occur until day 10. In one report, effusion was present in 22 percent of patients at 20 days and 8 percent at 30 days.

In most cases, the effusion is small and clinically insignificant; however, the effusion may be large, resulting in tamponade and hemodynamic instability and requiring urgent therapy with pericardiocentesis or reoperation. Postoperative anticoagulation may increase the risk of tamponade in patients who develop an effusion. In some patients, continuing pericardial inflammation over months leads to a thickened, fibrous pericardium and the signs and symptoms of constrictive pericarditis.

Transient constrictive pericarditis의 치료에 대한 내용입니다. For patients with newly diagnosed constrictive pericarditis who are hemodynamically stable and without evidence of chronic constriction, we suggest a trial of conservative management rather than pericardiectomy. Conservative management with anti-inflammatory agents can be continued for two to three months before proceeding with pericardiectomy, if indicated. Patients with markers of chronic constriction (eg, cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification) or signs of progressive systemic congestion (eg, dyspnea, unexplained weight gain, and/or a new or increased pleural effusion or ascites) should undergo earlier surgical intervention.

5. 50대/남자, 운동시 점차 심해지는 호흡곤란과 전신 부종으로 내원하였다. 7년 전 CABG를 시행 받은 병력이 있으며 CT에서 심장막 비후 소견이 보였다. O, X

1) 좌심방이 작다. → (X)
2) 흡기시 경정맥 팽창 → (O)
[U] Transthoracic echocardiography (TTE) is an essential diagnostic test in patients being evaluated for constrictive pericarditis. The American College of Cardiology/American Heart Association/American Society of Echocardiography guidelines and the European Society of Cardiology guidelines recommend the use echocardiography for the evaluation of all patients with suspected pericardial disease. Two-dimensional and M-mode echocardiography allow structural visualization while Doppler echocardiography provides hemodynamic information.

[U] Two-dimensional echocardiography – Two-dimensional echocardiography may reveal : 
• Dilatation of the inferior vena cava and hepatic veins (plethora) with absent or diminished inspiratory collapse.
•Moderate biatrial enlargement (severe enlargement is more compatible with restrictive cardiomyopathy).
•A sharp halt in ventricular diastolic filling (corresponding to the end of early rapid diastolic filling as noted on Doppler).
•Septal bounce with abrupt transient rightward movement of the interventricular septum.
•Hypermobile atrioventricular valves.
•An abnormal contour between the posterior LV and left atrial walls.

3) JVP 상에서 현저한 V파 → (X)
4) JVP에서 y파 하강의 둔화 → (X)

유착성 심장막염에서 주어지는 문제의 힌트는 prominent x descent, y descent 모두 존재입니다.  Prominent x descent는 atrial relaxation 부분이고 우측 사진(wedge Pr)의 early systolic dips입니다. Prominent y descent는 ventricular filling(tricuspid opening) 부분이고 우측 사진(PCW)의 early diastolic dips입니다. Ventricular filling 시작 이후에 ventricle은 갑자기 단단해진 pericardium에 제한되는데(rapid filling wave and plateau) 이것은  square root sign으로 나타납니다.

5) 흡기시 승모판 유입혈류 E파 속도 증가 → (X)
[U] Abnormal passive filling of the ventricles during early diastole – High E velocity of right and LV inflow is seen due to the abnormally rapid early diastolic filling associated with the combination of a small ventricular volume and rapid recoil. The early diastolic Doppler tissue velocity at the mitral annulus (E') is prominent (unless slowed by mitral annular calcification). The usually positive linear relation between E/E’ and left atrial pressure, which is useful for assessing left atrial pressure in cardiomyopathy, is reversed (annular paradox) in constrictive pericarditis.

[U] Pronounced respiratory variation in ventricular filling – Mitral inflow velocity falls as much as 25 to 40 percent and tricuspid velocity greatly increases (>40 to 60 percent) in the first cardiac cycle following inspiration. The respiratory variation in pulmonary venous flow is even more pronounced. These phenomena, which are manifestations of ventricular interdependence, are not present in either normal subjects or patients with restrictive cardiomyopathy. Increased respiratory variation of mitral inflow may be missing in patients with markedly elevated left atrial pressure, but can sometimes be elicited in such patients by preload reduction with semi-recumbent (rather than supine) positioning or diuretic administration. In an American Society of Echocardiography consensus statement, calculation of percentage respiratory variation for mitral and tricuspid inflow was standardized as:
[(expiration-inspiration)/expiration] X 100
Changes of 25 and 40 percent respectively are considered significant 

The 쉬운 내과, 문제 해설