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Postural hypotension following dorsal sympathectomy in coarctation of the aorta: Correction by resection of the coarctation
Postural
Hypotension
the Aorta:
Correction
John M. Evans, M.D., Washington, D. C.
Following
Dorsal
by Resection
Sympdectomy
in Coafctation
of
of the Coarctation
Irene Hsu, M.D.,
and Owen Gwathmey,
M.D.
In 1945, Grossi and Crafoord2 introduced operative resection for coarctation of the aorta, with relief of the hypertension in a great majority of the cases. Prior to this time sympathectomy had proved to be ineffective in lowering the blood pressure of these patients. In 1955, we had the opportunity to study a patient with coarctation of the aorta who had had a bilateral dorsal sympathectomy performed 10 years previously in another hospital. The hypertension was unaltered by the procedure, except that the patient developed severe postural hypotension. The response to resection of the coarctation was dramatic, with relief of both the hypertension and hypotension. Since, to our knowledge, these circumstances are unique, it seemed desirable to report the case in some detail. CASE
REPORT
A 34-year-old white woman was admitted to The George Washington University Hospital on Sept. 17, 1955, complaining of severe dizziness, fainting on standing, frequent severe headaches, and visual difficulty. Hypertension had been discovered in 1943, during the second trimester of pregnancy. A therapeutic abortion was advised, but was refused by the patient. She was placed at bed rest for the duration of the pregnancy and an uneventful cesarean section and tubal ligation were performed at term. In 1945, because of the persistence of hypertension, a bilateral dorsal sympathectomy was performed, with removal of the third through the twelfth thoracic ganglia. The hypertension was unaffected and severe postural hypotension developed. The systolic blood pressure levels ranged Syncope upon standing was unconfrom 180 to 200 mm. Hg when the patient was recumbent. trollable until she was fitted with a tight corset. During the next 10 years any attempt to get She continued to have episodic headaches and a along without the corset was unsuccessful. general lack of well-being. Antihypertensive drugs were used intermittently without response. The patient retailed that a pulsating swelling in the front of the neck had been brought to This swelling remained essentially unchanged over the her attention by a grade school teacher. years and was asymptomatic. On physical examination the patient was a well-developed and fairly well-nourished white while the patient was recumbent, woman who appeared to be her stated age. The blood pressure,
The
From the Departments of Medicine and Surgery, George Washington Univeretty School of Medicfne, Received for publication Feb. 21, 1969. 926
The Gieorge Wa&ington,
Weshington D.C.
Univerelty
Hospital.
EAE-“6’
POSTURAL
HYPOTENSION
AFTER
DORSAL
927
SYMPATHECTOMY
Several attempts were made was 200/125 mm. Hg in the arms and 180/110 mm. Hg in the legs. to record the blood pressure with the patient standing, without the corset, but prompt syncope occurred and a measurement could not be obtained. The most striking physical finding was a pulsating, tubular mass in the suprasternal notch. The mass was approximately 1.5 by 3 cm. in size. It appeared to originate beneath the right There were palpable pulsations and a systolic bruit over both suprasternocleidomastoid muscle. scapular regions. The heart was not enlarged and the lung fields were clear. A soft, Grade 2 systolic murmur was heard along the left sternal border. The murmur was not transmitted into the axilla and could not be heard in the back. The femoral pulsations were delayed and markedly diminished. The dorsalis pedis and posterior tibia1 pulses were not remarkable. The ocular fundi were normal. A heart film showed prominence of the left ventricle, without definite enlargement. The aortic knob was small. There was no rib notching. Electrocardiograms were found to be within normal limits. Studies of the blood and urine gave essentially normal results. An angiocardiogram revealed narrowing of the descending thoracic aorta approximately 2 cm. distal to the origin of the left subclavian artery (Figs. 1 and 2). The stenotic segment appeared to be 5 mm. in length. The innominate artery was tortuous and markedly dilated, The thyrocervical arterial trunk was elongated and dilated. On Sept. 21, 1955, a left thoracotomy was performed. The intercostal arteries were only The slightly enlarged. The coarctation was located at the level of the ligamentum arteriosum. The lumen coarctated segment was excised and a primary end-to-end anastomosis was performed. of the stenotic segment measured 3 mm. The postoperative course was essentially uneventful. The blood pressure in the arms reWhen the patient was first mained at 110 to 140 mm. Hg systolic and 70 to 90 mm. Hg diastolic. allowed out of bed on the eighth postoperative day, she promptly fainted as hypotension recurred. The amount of compression The tight-fitting corset was again used to control the hypotension. required to prevent postural attacks decreased progressively, and she was discharged on the twentieth hospital day in excellent condition. Six weeks following the operation the patient was able to abandon the corset, and has been free of postural symptoms ever since. At the present time she is completely well and working The suprasternal mass is only slightly apparent. full time. The blood pressure in the arms continues in the range of 110 to 140 mm. Hg systolic and 70 to 80 mm. Hg diastolic, and is slightly The soft systolic higher in the legs. The suprascapular pulsations and bruit have disappeared. murmur is still heard along the left sternal border. The femoral arterial pulsations are full and no longer delayed. The roentgenogram shows that the heart is now normal in size; the aortic knob is unchanged. COMMENT
In rection
discussing of
the
this
patient’s
coarctation
problem
seemed
to
preoperatively be
indicated.
it However,
was
agreed it
was
that not
corat
all
would be helped or aggravated by the procedure. The gradual loss of the long-standing, crippling syncope following operation was gratifying but difficult to understand. It is possible that the reduction in the arterial pressure gradient of blood flow from the upper to the lower parts of the body was contributory, the postural “run-off” being less sudden and less severe. That this is not the entire explanation is borne out by the recurrence of syncope in the immediate postoperative period when the blood pressure in the arms was essentially normal. The loss of the postural symptoms over a 6-week period suggests a gradual return of sympathetic tone in the splanchnic vascular bed. It is impossible to say how, or whether, this was related to resection of the coarctation. clear
whether
the
postural
hypotension
928
EVANS,
HSU,
AND
GWATHMEY
i\m. Heart J. June. 19.59
Volume 5 7 Number
6
POSTURAL
HYPOTENSION
AFTER
DORSAL
929
SYMPATHECTOMY
To our knowledge, the occurrence of hypotension in a setting such as that provided by our patient has not been reported previously in the medical literature. Glenn and associates3 reported a patient with coarctation involving the distal thoracic aorta and the celiac axis. Bilateral dorsal sympathectomy, performed 4 years earlier, had not influenced the hypertension but postural hypotension did not develop. Following resection of the coarctation and anastomosis of the splenic artery to the proximal aorta, the blood pressure fell to the normal range. In a review by Reifenstein and co-workers4 of 104 autopsied cases of coarctation of the aorta, mention is made of a few patients who had bilateral sympathectomy for hypertension. The follow-up was not discussed and reference to postural hypotension was not made. The lack of rib-notching in the roentgenograms of the chest in an adult is unusual. Reifenstein and associates4 however, reported absence of rib-notching in 25 per cent of their series. The enlarged thyrocervical trunk was atypical and probably served as the main proximal collateral channel, thus accounting for the absence of notching of the ribs. The pulsating mass in the suprasternal notch was confirmed by the angiocardiogram to be a buckled innominate artery. Sir John Parkinson and coworkers5 have emphasized that such a mass, in a young adult with hypertension, is strong presumptive evidence for coarctation of the aorta. With subsidence of the hypertension the swelling has almost disappeared. SUMMARY
A patient with coarctation of the aorta is, reported, in whom dorsal sympathectomy, 10 years earlier, left the arterial hypertension unchanged and caused severe postural hypotension. Both hypertension and postural hypotension were corrected by operative removal of the coarctation. REFERENCES
1. 2. 3. 4. 5.
Gross,
R. E., and Hufnagel, C. A.: Coarctation of garding Its Surgical Correction,. New England Crafoord, C., and Nylin, G.: Congemtal Coarctation ment, J. Thoracic Surg. 14:347, 1945. Glenn, F., Keefer, E. B., Speer, D. C., and Dotter, C. and Abdominal Aorta Immediately Proxrmal Obst. 94561, 1952. Reifenstein, C. H.., Levine, S. A., and Gross, R. D.: 104 Autopsred Cases of The Adult Type, AM. Parkinson, J., Bedford, D. E., and Almonds, S.: The Aneurysm, Brit. Heart J. 1:345, 1949.
the Aorta: Experimental Studies ReJ. Med. 233:287, 194.5. of the Aorta and Its Surgical TreatT.: Coarctation to the Celiac
of the Lower Thoracic Axis, Surg. Gynec. &
Coarctation of Aorta: HEART J. 33:146, 1947. Kinked Carotid Artery
A Review That
Simulates
of
Hypotension
the Aorta:
Correction
John M. Evans, M.D., Washington, D. C.
Following
Dorsal
by Resection
Sympdectomy
in Coafctation
of
of the Coarctation
Irene Hsu, M.D.,
and Owen Gwathmey,
M.D.
In 1945, Grossi and Crafoord2 introduced operative resection for coarctation of the aorta, with relief of the hypertension in a great majority of the cases. Prior to this time sympathectomy had proved to be ineffective in lowering the blood pressure of these patients. In 1955, we had the opportunity to study a patient with coarctation of the aorta who had had a bilateral dorsal sympathectomy performed 10 years previously in another hospital. The hypertension was unaltered by the procedure, except that the patient developed severe postural hypotension. The response to resection of the coarctation was dramatic, with relief of both the hypertension and hypotension. Since, to our knowledge, these circumstances are unique, it seemed desirable to report the case in some detail. CASE
REPORT
A 34-year-old white woman was admitted to The George Washington University Hospital on Sept. 17, 1955, complaining of severe dizziness, fainting on standing, frequent severe headaches, and visual difficulty. Hypertension had been discovered in 1943, during the second trimester of pregnancy. A therapeutic abortion was advised, but was refused by the patient. She was placed at bed rest for the duration of the pregnancy and an uneventful cesarean section and tubal ligation were performed at term. In 1945, because of the persistence of hypertension, a bilateral dorsal sympathectomy was performed, with removal of the third through the twelfth thoracic ganglia. The hypertension was unaffected and severe postural hypotension developed. The systolic blood pressure levels ranged Syncope upon standing was unconfrom 180 to 200 mm. Hg when the patient was recumbent. trollable until she was fitted with a tight corset. During the next 10 years any attempt to get She continued to have episodic headaches and a along without the corset was unsuccessful. general lack of well-being. Antihypertensive drugs were used intermittently without response. The patient retailed that a pulsating swelling in the front of the neck had been brought to This swelling remained essentially unchanged over the her attention by a grade school teacher. years and was asymptomatic. On physical examination the patient was a well-developed and fairly well-nourished white while the patient was recumbent, woman who appeared to be her stated age. The blood pressure,
The
From the Departments of Medicine and Surgery, George Washington Univeretty School of Medicfne, Received for publication Feb. 21, 1969. 926
The Gieorge Wa&ington,
Weshington D.C.
Univerelty
Hospital.
EAE-“6’
POSTURAL
HYPOTENSION
AFTER
DORSAL
927
SYMPATHECTOMY
Several attempts were made was 200/125 mm. Hg in the arms and 180/110 mm. Hg in the legs. to record the blood pressure with the patient standing, without the corset, but prompt syncope occurred and a measurement could not be obtained. The most striking physical finding was a pulsating, tubular mass in the suprasternal notch. The mass was approximately 1.5 by 3 cm. in size. It appeared to originate beneath the right There were palpable pulsations and a systolic bruit over both suprasternocleidomastoid muscle. scapular regions. The heart was not enlarged and the lung fields were clear. A soft, Grade 2 systolic murmur was heard along the left sternal border. The murmur was not transmitted into the axilla and could not be heard in the back. The femoral pulsations were delayed and markedly diminished. The dorsalis pedis and posterior tibia1 pulses were not remarkable. The ocular fundi were normal. A heart film showed prominence of the left ventricle, without definite enlargement. The aortic knob was small. There was no rib notching. Electrocardiograms were found to be within normal limits. Studies of the blood and urine gave essentially normal results. An angiocardiogram revealed narrowing of the descending thoracic aorta approximately 2 cm. distal to the origin of the left subclavian artery (Figs. 1 and 2). The stenotic segment appeared to be 5 mm. in length. The innominate artery was tortuous and markedly dilated, The thyrocervical arterial trunk was elongated and dilated. On Sept. 21, 1955, a left thoracotomy was performed. The intercostal arteries were only The slightly enlarged. The coarctation was located at the level of the ligamentum arteriosum. The lumen coarctated segment was excised and a primary end-to-end anastomosis was performed. of the stenotic segment measured 3 mm. The postoperative course was essentially uneventful. The blood pressure in the arms reWhen the patient was first mained at 110 to 140 mm. Hg systolic and 70 to 90 mm. Hg diastolic. allowed out of bed on the eighth postoperative day, she promptly fainted as hypotension recurred. The amount of compression The tight-fitting corset was again used to control the hypotension. required to prevent postural attacks decreased progressively, and she was discharged on the twentieth hospital day in excellent condition. Six weeks following the operation the patient was able to abandon the corset, and has been free of postural symptoms ever since. At the present time she is completely well and working The suprasternal mass is only slightly apparent. full time. The blood pressure in the arms continues in the range of 110 to 140 mm. Hg systolic and 70 to 80 mm. Hg diastolic, and is slightly The soft systolic higher in the legs. The suprascapular pulsations and bruit have disappeared. murmur is still heard along the left sternal border. The femoral arterial pulsations are full and no longer delayed. The roentgenogram shows that the heart is now normal in size; the aortic knob is unchanged. COMMENT
In rection
discussing of
the
this
patient’s
coarctation
problem
seemed
to
preoperatively be
indicated.
it However,
was
agreed it
was
that not
corat
all
would be helped or aggravated by the procedure. The gradual loss of the long-standing, crippling syncope following operation was gratifying but difficult to understand. It is possible that the reduction in the arterial pressure gradient of blood flow from the upper to the lower parts of the body was contributory, the postural “run-off” being less sudden and less severe. That this is not the entire explanation is borne out by the recurrence of syncope in the immediate postoperative period when the blood pressure in the arms was essentially normal. The loss of the postural symptoms over a 6-week period suggests a gradual return of sympathetic tone in the splanchnic vascular bed. It is impossible to say how, or whether, this was related to resection of the coarctation. clear
whether
the
postural
hypotension
928
EVANS,
HSU,
AND
GWATHMEY
i\m. Heart J. June. 19.59
Volume 5 7 Number
6
POSTURAL
HYPOTENSION
AFTER
DORSAL
929
SYMPATHECTOMY
To our knowledge, the occurrence of hypotension in a setting such as that provided by our patient has not been reported previously in the medical literature. Glenn and associates3 reported a patient with coarctation involving the distal thoracic aorta and the celiac axis. Bilateral dorsal sympathectomy, performed 4 years earlier, had not influenced the hypertension but postural hypotension did not develop. Following resection of the coarctation and anastomosis of the splenic artery to the proximal aorta, the blood pressure fell to the normal range. In a review by Reifenstein and co-workers4 of 104 autopsied cases of coarctation of the aorta, mention is made of a few patients who had bilateral sympathectomy for hypertension. The follow-up was not discussed and reference to postural hypotension was not made. The lack of rib-notching in the roentgenograms of the chest in an adult is unusual. Reifenstein and associates4 however, reported absence of rib-notching in 25 per cent of their series. The enlarged thyrocervical trunk was atypical and probably served as the main proximal collateral channel, thus accounting for the absence of notching of the ribs. The pulsating mass in the suprasternal notch was confirmed by the angiocardiogram to be a buckled innominate artery. Sir John Parkinson and coworkers5 have emphasized that such a mass, in a young adult with hypertension, is strong presumptive evidence for coarctation of the aorta. With subsidence of the hypertension the swelling has almost disappeared. SUMMARY
A patient with coarctation of the aorta is, reported, in whom dorsal sympathectomy, 10 years earlier, left the arterial hypertension unchanged and caused severe postural hypotension. Both hypertension and postural hypotension were corrected by operative removal of the coarctation. REFERENCES
1. 2. 3. 4. 5.
Gross,
R. E., and Hufnagel, C. A.: Coarctation of garding Its Surgical Correction,. New England Crafoord, C., and Nylin, G.: Congemtal Coarctation ment, J. Thoracic Surg. 14:347, 1945. Glenn, F., Keefer, E. B., Speer, D. C., and Dotter, C. and Abdominal Aorta Immediately Proxrmal Obst. 94561, 1952. Reifenstein, C. H.., Levine, S. A., and Gross, R. D.: 104 Autopsred Cases of The Adult Type, AM. Parkinson, J., Bedford, D. E., and Almonds, S.: The Aneurysm, Brit. Heart J. 1:345, 1949.
the Aorta: Experimental Studies ReJ. Med. 233:287, 194.5. of the Aorta and Its Surgical TreatT.: Coarctation to the Celiac
of the Lower Thoracic Axis, Surg. Gynec. &
Coarctation of Aorta: HEART J. 33:146, 1947. Kinked Carotid Artery
A Review That
Simulates
of