Cirrhosis of the Liver Simulating Congenital Cyanotic Heart Disease: Results

Category: Heart Disease | Tags: arteriovenous fistulas, cyanotic heart disease, pulmonary, pulmonary angiography

The results of cardiac catheterization, angiography, and biopsy are shown in Table 1 and Figures 1 to 6. Eleven of the patients were men. All patients were between seven and 21 years of age. All of them showed severe cyanosis, gross clubbing, and grade 2-3 effort intolerance of one to six years’ duration. Eight patients complained of a lump in the abdomen, and one complained of soft swelling on the right forearm, swelling of wrists and ankles, and exhibited wing flap tremors. There was history of hematemesis in one. There was no history of alcoholism, protein malnutrition, or umbilical sepsis in any case.

Every patient had severe cyanosis, gross clubbing, warm hands, wide pulse pressure, a grade 2/6 ejection systolic murmur at the pulmonary area, and a normally split second heart sound with normal aortic and pulmonary components. The spleen was palpably enlarged. The liver was palpable one to three finger breadths below the costal margin in 18 cases. One patient had marked swelling of the ankles and wrists due to osteoarthropathy. He also had a hemangioma on the lateral aspect of the upper part of the right fore arm. He exhibited marked wing flap tremor of the outstretched hands, but there was no Kayser-Fleischer ring nor any other neurologic abnormality. Three patients showed arterial spiders and talangiectasia on the chest, back, and arms. One of these three patients showed a caput medusae and another a few engorged veins on the abdomen. Reading here

There was polycythemia in 18. Spectroscopic examination of blood revealed no abnormality. The liver function test results were slightly abnormal in 17 patients. The serum globulin was raised in 17. In five patients in whom plasma electrophoresis was performed, the rise was largely due to gamma-globulin. Sulfobromophthalein (Bromsulfalein) retention was increased and flocculation tests were positive in 15. Barium swallow demonstrated esophageal varices in eight cases. Histologic examination of needle biopsy specimens of liver in 19 cases and open biopsy in one case revealed portal cirrhosis of the liver.
Table 1-Catheterization Data as Pressure, mmHg, and Oxygen Saturation (%)

CaseNo. PA RV RA SVC IVC SAt PVt
1 20/7 (65) 25/3 (65) 3 (63) 3 (68) 3 (60) 110/60 (60) (64)
2 25/5 (55) 25/0 (55) 5 (53) 5 (58) 5 (50) 130/65 (69) (78)
3 20/5 (58) 20/2 (58) 5 (57) 5 (60) 5 (53) 120/60 (72) (74)
4 20/6 (64) 25/3 (64) 3 (63) 3 (62) 3 (64) 110/55 (75)
5 28/8 (62) 28/6 (62) 6 (61) 6 (63) 6 (58) 100/50 (79)
6 25/6 (58) 25/0 (58) 4 (58) 4 (60) 4 (53) 110/45 (78)
7 26/8 (62) 26/4 (62) 4 (60) 4 (58) 4 (63) 110/50 (82)
8 30/12 (64) 30/6 (64) 4 (62) 4 (62) 4 (66) 110/50 (78)
9 2(V8 (65) 20/4 (65) 4 (63) 4 (65) 4 (60) 110/60 (60)
10 25/5 (58) 25/4 (58) 4 (56) 4 (56) 4 (60) 120/60 (70) (72)
11 28/12 (62) 28/6 (62) 6 (61) 6(61) 6(65) 110/60 (75)
12 30/12 (60) 30/6 (60) 6 (58) 6 (58) 6 (63) 130/65 (72)
13 25/10 (62) 25/6 (62) 6 (60) 6 (60) 6 (64) 120/60 (75)
14 20/7 (64) 20/5 (62) 5 (62) 5 (62) 5 (61) 110/60 (66)
15 28/8 (62) 28/8 (62) 6 (62) 6 (63) 6 (64) 100/50 (77) (78)
16 25/12 (65) 25/5 (65) 5 (64) 5 (67) 5(68) 130/65 (68)
17 30/12 (60) 30/6 (60) 6 (58) 6 (58) 6 (63) 110/60 (70)
18 25/10 (55) 25/4 (55) 4 (55) 4 (55) 4 (58) 120/60 (68) (70)
19 20/6 (64) 20/3 (64) 3 (63) 3 (62) 3 (64) 110/75 (72)
20 30/15 (62) 30/6 (62) 6 (62) 6 (62) 6(64) 110/60 (72)

 

Figure 1. Liver biopsy (case 1) showing irregular areas of fibrous tissue traversing the parenchymatous tissue, pseudolobulation, regenerating nodules. (From Hansoti RC, Shah RC. Cirrhosis of liver simulating congenital cyanotic heart disease. Circulation 1966; 33:71-7, by permission.)

Figure 1. Liver biopsy (case 1) showing irregular areas of fibrous tissue traversing the parenchymatous tissue, pseudolobulation, regenerating nodules. (From Hansoti RC, Shah RC. Cirrhosis of liver simulating congenital cyanotic heart disease. Circulation 1966; 33:71-7, by permission.)

Figure 2. The pulmonary vasculature appears blotchy on main pulmonary artery angiogram (group A). (From Hansoti RC, Shah RC. Cirrhosis of liver simulating congenital cyanotic heart disease. Circulation 1966; 33:71-7, by permission.)

Figure 2. The pulmonary vasculature appears blotchy on main pulmonary artery angiogram (group A). (From Hansoti RC, Shah RC. Cirrhosis of liver simulating congenital cyanotic heart disease. Circulation 1966; 33:71-7, by permission.)

Figure 3. Selective right pulmonary artery angiography showing multiple tiny pulmonary arteriovenous fistulas (group A).

Figure 3. Selective right pulmonary artery angiography showing multiple tiny pulmonary arteriovenous fistulas (group A).

Figure 4. Main pulmonary artery angiography from a case (group B) showing no convincing evidence of pulmonary arteriovenous fistulas.

Figure 4. Main pulmonary artery angiography from a case (group B) showing no convincing evidence of pulmonary arteriovenous fistulas.

Figure 5. Peripheral vein contrast echocardiography studies in a case (group B) showing passage of contrast material in the left atrium and left ventricle four to five cycles after its appearance in the right heart chambers. Abbreviations: RV, right ventricle; RA, right atrium; LV, left ventricle; LA, left atrium.

Figure 5. Peripheral vein contrast echocardiography studies in a case (group B) showing passage of contrast material in the left atrium and left ventricle four to five cycles after its appearance in the right heart chambers. Abbreviations: RV, right ventricle; RA, right atrium; LV, left ventricle; LA, left atrium.

Figure 6. Lung biopsy specimen showing collections of arteriolar spaces filled with blood and crowded in certain areas.

Figure 6. Lung biopsy specimen showing collections of arteriolar spaces filled with blood and crowded in certain areas.