IN the Cases of Primary Pulmonary Hypertension (PPH)
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Arrhythmias in Primary Pulmonary Hypertension Nariaki KANEMOTO,M.D. and Hiroshi SASAMOTO,M.D. SUMMARY Through the examination of 171 electrocardiograms (ECG) of 101 patients with primary pulmonary hypertension (PPH), the following con- clusions were obtained. Arrhythmias were found in 27 (26.7%) of the 101 patients. Among the surviving cases, arrhythmias were found in 8 (17.8%) out of 45 pa- tients, but among the deceased cases, the figure was higher, 19 (33.9%) out of 56. There were instances of more than one kind of arrhythmias in the same patient and a total of 34 types of arrhythmias were noted. The main types of arrhythmias were sinus tachycardia (13 cases), sinus bradycardia (6 cases), and first degree A-V block (5 cases), which ac- counted for 70% of the total. Sinus tachycardia was observed in only 2 of the survivors but in 11 of the deceased cases, which showed a sig- nificant difference (p<0.05). First degree A-V block were all induced by digitalis. Wenckebach type of second degree A-V block was noted in only 1 case and it was also induced by digitalis. Since severe attacks of ventricular arrhythmias were very rare in these cases with PPH, it is difficult to consider the Adams-Stokes syndrome as the cause of syncopal attacks or sudden death. However, it is necessary to further investigate nodal functions by means of continuous ECG monitoring, the overdrive suppression test and His bundle ECGs. Additional Indexing Words: Syncopal attacks Adams-Stokes syndrome Major supraventricular arrhythmias Ventricular arrhythmias Sudden death N the cases of primary pulmonary hypertension (PPH), the major subjec- I tive symptoms are palpitation, dyspnea, and syncope, and there have also been many cases of sudden death.1) Since there is a possibility that these symptoms occur because of electrical failure, electrocardiographic examina- tions should be done. The authors have already reported various electro- cardiographic findings except those concerning arrhythmias.2) Therefore, this paper deals with an investigation of arrhythmias in PPH. From the Department of Internal Medicine, School of Medicine, Tokai University , Boseidai, Isehara, Kanagawa 259-11, Japan. This study was supported in part by a Research Grant from the Intractable Diseases Division , Public Health Bureau, Ministry of Health and Welfare, Japan. Received for publication February 21, 1979. Manuscript revised March 23, 1979. 765 Jap. Heart J. 766 KANEMOTO AND SASAMOTO November, 1979 Table I. Proposed Guideline for the Diagnosis of Primary Pulmonary Hypertension Primary pulmonary hypertension is a clinical diagnostic name of pulmonary hypertension of unknown cause. It is required: To confirm pulmonary arterial (pre-capillary) hypertension and/or right ventricular hy- pertrophy due to pulmonary hypertension. To ascertain that the pulmonary hypertension is to be primary. A. Symptoms and signs that suggest pulmonary arterial hypertension and/or right ventricular hypertrophy: I. Cardinal symptoms and signs 1. dyspnea 2. easy fatigability 3. retrosternal pain (pulmonary hypertensive pain) or syncope on exertion 4. right ventricular heave 5. accentuation of P2 and S4, diastolic murmur at pulmonic area, systolic regurgitant murmur at tricuspid area II. Laboratory data 1. prominence of the central pulmonary artery trunk and decreased peripheral pul- monary vasculature on chest roentgenograms 2. right ventricular hypertrophy on electrocardiogram 3. normal or slightly disturbed restrictive ventilatory function (almost normal SaO2) 4. right heart catheterization i) increased pulmonary arterial pressure (mean pressure is 25mmHg or more) ii) normal pulmonary capillary wedge pressure (12mmHg or less) 5. increased 'a' wave in jugular venous pressure B. Procedure suggesting that pulmonary hypertension is to be primary: There are some cases of primary pulmonary hypertension that show increased rate of erythrocyte sedimentation rate, increased value of ƒÁ-globulin, immunological disorders, rarely arthritis, Raynaud's phenomenon and splenomegaly. After recognizing no existence of primary or congenital cardiopulmonary disease, and complication of liver cirrhosis, the following histological changes should be observed: III. Histological findings Pulmonary vascular lesions that show medial hypertrophy, concentric intimal fibrosis, necrotizing arteritis, and plexiform lesions. IV. Diseases to be excluded Since the following diseases may cause pulmonary hypertension succeeded by right ventricular hypertrophy, it is necessary to exclude:1 . Diseases primarily affecting air passage of the lung and the alveoli chronic bronchitis, bronchial asthma, emphysema, pulmonary fibrosis and pneu- monitis of various origins, pulmonary granulomatosis (e.g. sarcoidosis, berylliosis, histiocytosis, tuberculosis), collagen diseases, infectious diseases of the lung, malig- nant tumors of the lung, alveolar microlithiasis, congenital cystic disease of the lung, pulmonary resection, hypoxia of severe degree (e.g. mountain sickness and chronic obstructive diseases of upper respiratory airway.) (Continued to the next page) Vol.20 No.6 ARRHYTHMIAS IN PRIMARY PULMONARY HYPERTENSION 767 (Continued) 2. Diseasesprimarily affecting the movementsof the thoraciccage kyphoscoliosisand other thoracicdeformities, thoracoplasty, pleural fibrosis, chronic neuromuscularweakness (e.g. poliomyelitis),obesity with alveolar hypoventilation, idiopathicalveolar hypoventilation. 3. Diseasesprimarily affecting the pulmonaryvasculature pulmonarythromboembolism, collagen diseases, and other arteritis, schistosomiasis, sicklecell anemia,pressures on main pulmonaryarteries and veinsby mediastinal tumors,aneurysm, granuloma or fibrosisand pulmonaryveno-occlusive disease. 4. Diseasesprimarily affecting the left ventricle valvular heart diseases(especially mitral stenosis)and left heart failure. 5. Congenitalheart diseases atrial septal defect, ventricularseptal defect, patent ductusarteriosus and others. [Criteria for the diagnosis] 1. Definitecase To meet items more than half of I and II, and conditionsof III and IV. 2. Probablecase To meet items more than half of I and II, and condition of IV but without histologicalexamination. SUBJECTSAND METHODS The subjects used in this study were 101 cases of PPH with 171 electrocardio- grams (ECGs). The ECG copies were obtained from hospitals throughout Japan which had reported these cases. These 101 cases included 28 males (of whom 16 have died) and 73 females (of whom 40 have died) ranging in age from 9 to 66 years with a mean age of 30.8 years. By the time of this study, living patients survived 12 to 96 months with an average of 30.5 months. In the dead patients, causes of death were clarified in 38 patients, among which 23 (60.5%) died of right heart failure, 12 (31.6%) died suddenly, 2 died of massive hemoptysis, and 1 pneumonia. These patients died 1 day to 24 months with an average of 7.2 months after the recording of ECGs. The ECGs were obtained with the standard 12 leads, a paper speed of 25 mm/sec and a sensitivity of 1mV=10mm or 5mm. In most cases, there were recordings of 5 to 10sec per lead. These ECGs were investigated for arrhythmias in all of the patients and in the surviving and the deceased groups. Among 32 patients, 2 to 7 ECGs for each patient were obtained but the same arrhythmia found in the same case was counted as only one. The diagnosis of PPH was made independently in each hospital in accordance with the proposed guideline for the diagnosis of PPH by the Ministry of Health and Welfare PPH Research Committee.2) RESULTS Out of the 101 subjects, 27 (26.7%) were found to have some type of arrhythmia. In the surviving group, there were only 8 out of 45 cases (17.8%) Jap. Heart J. N 768 KANEMOTO AND SASAMOTO ovember, 1979 Table II. Occurrence of Arrhythmias Table III. Types of Arrhythmias *p<0 .05 with arrhythmias, while in the deceased group, there were many more cases of arrhythmias, 19 out of 56 (33.9%). There were instances where arrhyth- mias occurred more than once in the same patient and the total number of incidence of arrhythmias was 34 (Table II). Table III shows the types and frequencies of the arrhythmias. There were 13 cases with sinus tachycardia (R-R interval of 0.48 to 0.59sec) in- cluding 11 of the deceased cases which was a significant majority (p<0.05). Fig. 1 shows the ECG of a 35-year-old female who came to the hospital suffering mainly from dyspnea and anasarca. This patient died of right heart failure 2 months later. Therefore, it appears that sinus tachycardia seen in cases of PPH is often caused by right heart failure and is an ominous sign of poor prognosis. Conversely, sinus bradycardia (R-R interval of 1.04 to 1.32sec) was observed in 6 cases, most of them survived. Fig. 2 shows the ECG of a 24- year-old male who survived. There are atrioventricular (A-V) junctional escaped beats due to sinus bradycardia and arrhythmia. First degree A-V block was found in 5 cases, 4 of them died. All of Vol.20 No.6 ARRHYTHMIAS IN PRIMARY PULMONARY HYPERTENSION 769 Fig. 1. Electrocardiogram (H.S., 35 years old, female) showing sinus tachycardia at a rate of 104 beats per minute. Fig. 2. Electrocardiogram (H.O., 24 years old, male) showing sinus bradycardia and occasional A-V junctional escaped beats (with the courtesy of Prof. Dr. Hara). Jap. Heart J. N 770 KANEMOTO AND SASAMOTO ovember, 1979 these cases were given digitalis for right heart failure. The PQ interval was 0.22 to 0.30sec. Second degree A-V block was seen in only 1 case. Fig. 3 (A) shows the ECG of a 32-year-old female on digitalis and diuretics for right heart failure. Sinus tachycardia and Wenckebach type of second degree A-V block were found. The second degree A-V block disappeared 10 days after the reduction of digitalis (Fig. 3 (B)). Atrial fibrillation and atrial flutter were observed in 1 case each. Premature beats, however, were rare. Supraventricular premature beats were found in only 3 cases and ventricular premature beats in only 1 case. Fig. 4 shows the ECG of a 41-year-old female with frequent supraven- tricular premature beats causing parasystole and a ventricular premature beat.