7
Volume 69 March 1976
223
Clinical Section President Ian Burn FRCS
Meeting 14 March 1975 (continuedfrom January 1976 'Proceedings', p 62)
Cases Bidirectional Ventricular Tachycardia in Familial Hypokalasmic Periodic Paralysis W A Stubbs' BSC MRCP (Hammersmith Hospital, London W12) Mrs V L, age 31. Housewife History: Aged 2 years, an irregular pulse was noted at routine medical examination. She has noted palpitations throughout her life. From age 28, she had episodes of periodic paralysis of varying severity lasting up to one week, accompanied by myalgia. Paralysis was most marked in the proximal muscles but spared the facial and respiratory muscles, was precipitated by tension and fever, prevented by mild exercise and occurred two or three times a year. Aged 29 years she had her first blackout followed by several similar attacks, three of which were documented as ventricular fibrillation. Family history: Mother died aged 37 with 'heart failure'; further family history was not available. The patient has a son aged 5 years with sensorineural deafness, a son aged 3 years with nephrolithiasis, and a daughter aged 4 years with a suspected mild myopathy; all 3 have normal ECG. On examination: Normal except for an irregular pulse and generalized muscle weakness most marked in the proximal muscles. Investigations: Chest X-ray normal. ECG: bidirectional ventricular tachycardia with occasional sinus beats. ECG taken during a paralytic episode showed evidence of hypokakemia of greater severity than the serum potassium suggested. Intracardiac stimulation studies showed sinus and atrioventricular node disease with alternating ventricular foci, producing bidirectional ventricular tachycardia. Electromyogram normal. Skeletal muscle biopsy showed vacuolation. Full blood count and ESR normal. Serum creatine, phosphokinase, aspartate amino trans'Present Address: Department of Chemical Pathology, Southampton General Hospital, Tremona Road, Southampton, S09 4XY
ferase, alkaline phosphatase, calcium, phosphate, bilirubin, cholesterol and uric acid all normal. Serum potassium 3.1 mEq/1. Urea and electrolytes otherwise normal. Treatment: A permanent epicardial demand pacemaker was inserted. Cardiac muscle was seen to be extremely thin and atrophic at operation. The ventricular extrasystoles were suppressed initially with intravenous lignocaine and subsequently oral mexiletene. Potassium supplements and amiloride were also given. Follow-up: Initially the ECG showed alternating paced and junctional beats but at six months showed sinus rhythm. The patient remains well and free from blackouts. Discussion Four cases of sustained cardiac arrhythmia in familial periodic paralysis have been reported, of which only one occurred in a patient with hypokalemic periodic paralysis (Klein et al. 1963, Levitt et al. 1972, Lisak et al. 1972). Rarely an arrhythmia may occur transiently in induced attacks of periodic paralysis (Gass et al. 1948, Scherf & Schott 1973). The sustained arrhythmia is unrelated to attacks of paralysis and consists of ventricular extrasystoles or tachycardia, frequently bidirectional in nature. It has previously been found resistant to treatment, although quinidine may reduce the frequency of the arrhythmia and acetazolamide prevent sudden death (Klein et al. 1963, Levitt et al. 1972, Lisak et al. 1972). Our patient was converted from ventricular fibrillation with DC shock and showed good response to lignocaine and mexiletene. As with the cases of Klein et al. (1963) and Lisak et al. (1972), the ECG was improved by both oral and intravenous potassium but did not correlate with changes in the serum potassium. We also believe the insertion of an epicardial pacemaker to be of particular value in the management of certain arrhythmias, such as this, by reducing the risk of
drug-induced asystole.
224
Proc. roy. Soc. Med. Volume 69 March 1976
REFERENCES Gass H, Cherkassky M & Savitsky N (1948) Medicine (Baltimore) 27, 105-137 Klein R, Ganelin R, Marks J F, Usher P & Richards C (1963) Journal ofPediatrics 62, 371-385 Levitt L P. Rose L I & Dawson D M (1972) New England Journal ofMedicine 286, 253-254 Lisak R P, Lebau J, Tucker S H & Rowland L P (1972) Neurology (Minneapolis) 22, 810-815 Scherf D & Schott A (1973) Extrasystoles and Allied Arrhythmias. 2nd edn. Heinemann Medical, London; p 768
The following cases were also presented: Recovery of Eleven Years' Bone Marrow Aplasia Following a Septic2emic Episode Dr Katharine Murray (for Dr R P K Coe) (West Middlesex Hospital, Isleworth)
Controlled Environment Treatment of (Edema and Ischsemia on the Upper Limb Mr J E Hale (Queen Mary's Hospital, Roehampton, London SW15)
Meeting 21 May 1975 at Charing Cross Hospital, London W6
Cases Phleochromocytoma Presenting in Pregnancy G B Coombes FRCS FRCSEd (for Ian Burn FRCS) (Charing Cross Hospital, London W6)
8 herself stopped in January 1975. Blackouts recommenced. Admitted 9.2.75 for localizing investigations and surgery. Investigations: IVP normal, selective renal arteriography normal. Retroperitoneal air insufflation (Fig 1) outlined a tumour behind the upper pole of left kidney. Further investigations were refused. Operation (19.2.75): Phwochromocytoma located where expected and successfully removed (Fig 2). No contralateral tumour. Histologically benign. Patient made a full recovery. Discussion Phxochromocytoma during pregnancy is highly dangerous for both mother and fetus. The previously reported case from this hospital resulted in death of both (Coden 1972). Walker (1964) reviewed 68 cases with a maternal and fetal mortality of 50%, although a-adrenergic blockade was not in use. More recently Smith (1973) reported 3 cases: one was successfully managed under phenoxybenzamine control, one resulted in fetal death from cerebral haemorrhage, and the third in maternal death; only the first case was controlled with phenoxybenzamine. We advised that if a phaeochromocytoma were diagnosed during early pregnancy phenoxybenzamine should be started and a therapeutic termination then carried out; attempts should next be made to localize the tumour using the radiological techniques advocated by Sutton (1975), before removing it surgically. Should the diagnosis not be made until the second or third trimester, the pregnancy should be allowed to continue
Table 1 Mrs J L, aged 39. Jamaican History: September 1973, presented to the obstetric department 13 weeks pregnant, gravida 10, para 6; complained of headaches; dizziness, feeling confused, occasional palpitations and short blackouts. Admitted for investigation. Over the next five months routine blood pressure recordings showed a maximum of 140/90 mmHg except during severe attacks, when a rise to 170/100 mmHg was recorded. These attacks were associated with visual aurae, seen as flashing lights. No family history of pheochromocytoma. Investigations (November 1973--February 1974): Results of tests (Tables 1 and 2) indicated the presence of a ph2ochromocytoma; her pregnancy was allowed to proceed under phenoxybenzamine control, and good palliation was obtained. 20.3.74: Labour induced, forceps delivery of a healthy child, all under phenoxybenzamine control. The patient refused to consider any further investigations or treatment but consented to continue with phenoxybenzamine, which she
Results of biochemical screening, November 1973-February 1974 No. qf
Urinary VMA (3methoxy-4hydroxymandelic acid) (mg/24 h) Metadrenaline and normetadrenaline (as metadrenaline, mg/24 h) Total urinary catecholamines (noradrenaline, ,ug/24 h)
observaMean Range tions 11.5 13 8.1-16.2
7
2.5
5
1668
Normal
Volume 69 March 1976
223
Clinical Section President Ian Burn FRCS
Meeting 14 March 1975 (continuedfrom January 1976 'Proceedings', p 62)
Cases Bidirectional Ventricular Tachycardia in Familial Hypokalasmic Periodic Paralysis W A Stubbs' BSC MRCP (Hammersmith Hospital, London W12) Mrs V L, age 31. Housewife History: Aged 2 years, an irregular pulse was noted at routine medical examination. She has noted palpitations throughout her life. From age 28, she had episodes of periodic paralysis of varying severity lasting up to one week, accompanied by myalgia. Paralysis was most marked in the proximal muscles but spared the facial and respiratory muscles, was precipitated by tension and fever, prevented by mild exercise and occurred two or three times a year. Aged 29 years she had her first blackout followed by several similar attacks, three of which were documented as ventricular fibrillation. Family history: Mother died aged 37 with 'heart failure'; further family history was not available. The patient has a son aged 5 years with sensorineural deafness, a son aged 3 years with nephrolithiasis, and a daughter aged 4 years with a suspected mild myopathy; all 3 have normal ECG. On examination: Normal except for an irregular pulse and generalized muscle weakness most marked in the proximal muscles. Investigations: Chest X-ray normal. ECG: bidirectional ventricular tachycardia with occasional sinus beats. ECG taken during a paralytic episode showed evidence of hypokakemia of greater severity than the serum potassium suggested. Intracardiac stimulation studies showed sinus and atrioventricular node disease with alternating ventricular foci, producing bidirectional ventricular tachycardia. Electromyogram normal. Skeletal muscle biopsy showed vacuolation. Full blood count and ESR normal. Serum creatine, phosphokinase, aspartate amino trans'Present Address: Department of Chemical Pathology, Southampton General Hospital, Tremona Road, Southampton, S09 4XY
ferase, alkaline phosphatase, calcium, phosphate, bilirubin, cholesterol and uric acid all normal. Serum potassium 3.1 mEq/1. Urea and electrolytes otherwise normal. Treatment: A permanent epicardial demand pacemaker was inserted. Cardiac muscle was seen to be extremely thin and atrophic at operation. The ventricular extrasystoles were suppressed initially with intravenous lignocaine and subsequently oral mexiletene. Potassium supplements and amiloride were also given. Follow-up: Initially the ECG showed alternating paced and junctional beats but at six months showed sinus rhythm. The patient remains well and free from blackouts. Discussion Four cases of sustained cardiac arrhythmia in familial periodic paralysis have been reported, of which only one occurred in a patient with hypokalemic periodic paralysis (Klein et al. 1963, Levitt et al. 1972, Lisak et al. 1972). Rarely an arrhythmia may occur transiently in induced attacks of periodic paralysis (Gass et al. 1948, Scherf & Schott 1973). The sustained arrhythmia is unrelated to attacks of paralysis and consists of ventricular extrasystoles or tachycardia, frequently bidirectional in nature. It has previously been found resistant to treatment, although quinidine may reduce the frequency of the arrhythmia and acetazolamide prevent sudden death (Klein et al. 1963, Levitt et al. 1972, Lisak et al. 1972). Our patient was converted from ventricular fibrillation with DC shock and showed good response to lignocaine and mexiletene. As with the cases of Klein et al. (1963) and Lisak et al. (1972), the ECG was improved by both oral and intravenous potassium but did not correlate with changes in the serum potassium. We also believe the insertion of an epicardial pacemaker to be of particular value in the management of certain arrhythmias, such as this, by reducing the risk of
drug-induced asystole.
224
Proc. roy. Soc. Med. Volume 69 March 1976
REFERENCES Gass H, Cherkassky M & Savitsky N (1948) Medicine (Baltimore) 27, 105-137 Klein R, Ganelin R, Marks J F, Usher P & Richards C (1963) Journal ofPediatrics 62, 371-385 Levitt L P. Rose L I & Dawson D M (1972) New England Journal ofMedicine 286, 253-254 Lisak R P, Lebau J, Tucker S H & Rowland L P (1972) Neurology (Minneapolis) 22, 810-815 Scherf D & Schott A (1973) Extrasystoles and Allied Arrhythmias. 2nd edn. Heinemann Medical, London; p 768
The following cases were also presented: Recovery of Eleven Years' Bone Marrow Aplasia Following a Septic2emic Episode Dr Katharine Murray (for Dr R P K Coe) (West Middlesex Hospital, Isleworth)
Controlled Environment Treatment of (Edema and Ischsemia on the Upper Limb Mr J E Hale (Queen Mary's Hospital, Roehampton, London SW15)
Meeting 21 May 1975 at Charing Cross Hospital, London W6
Cases Phleochromocytoma Presenting in Pregnancy G B Coombes FRCS FRCSEd (for Ian Burn FRCS) (Charing Cross Hospital, London W6)
8 herself stopped in January 1975. Blackouts recommenced. Admitted 9.2.75 for localizing investigations and surgery. Investigations: IVP normal, selective renal arteriography normal. Retroperitoneal air insufflation (Fig 1) outlined a tumour behind the upper pole of left kidney. Further investigations were refused. Operation (19.2.75): Phwochromocytoma located where expected and successfully removed (Fig 2). No contralateral tumour. Histologically benign. Patient made a full recovery. Discussion Phxochromocytoma during pregnancy is highly dangerous for both mother and fetus. The previously reported case from this hospital resulted in death of both (Coden 1972). Walker (1964) reviewed 68 cases with a maternal and fetal mortality of 50%, although a-adrenergic blockade was not in use. More recently Smith (1973) reported 3 cases: one was successfully managed under phenoxybenzamine control, one resulted in fetal death from cerebral haemorrhage, and the third in maternal death; only the first case was controlled with phenoxybenzamine. We advised that if a phaeochromocytoma were diagnosed during early pregnancy phenoxybenzamine should be started and a therapeutic termination then carried out; attempts should next be made to localize the tumour using the radiological techniques advocated by Sutton (1975), before removing it surgically. Should the diagnosis not be made until the second or third trimester, the pregnancy should be allowed to continue
Table 1 Mrs J L, aged 39. Jamaican History: September 1973, presented to the obstetric department 13 weeks pregnant, gravida 10, para 6; complained of headaches; dizziness, feeling confused, occasional palpitations and short blackouts. Admitted for investigation. Over the next five months routine blood pressure recordings showed a maximum of 140/90 mmHg except during severe attacks, when a rise to 170/100 mmHg was recorded. These attacks were associated with visual aurae, seen as flashing lights. No family history of pheochromocytoma. Investigations (November 1973--February 1974): Results of tests (Tables 1 and 2) indicated the presence of a ph2ochromocytoma; her pregnancy was allowed to proceed under phenoxybenzamine control, and good palliation was obtained. 20.3.74: Labour induced, forceps delivery of a healthy child, all under phenoxybenzamine control. The patient refused to consider any further investigations or treatment but consented to continue with phenoxybenzamine, which she
Results of biochemical screening, November 1973-February 1974 No. qf
Urinary VMA (3methoxy-4hydroxymandelic acid) (mg/24 h) Metadrenaline and normetadrenaline (as metadrenaline, mg/24 h) Total urinary catecholamines (noradrenaline, ,ug/24 h)
observaMean Range tions 11.5 13 8.1-16.2
7
2.5
5
1668
Normal
