Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Coarctation of the aorta Barbara L. Klein & Abdulmassih S. Iskandrian To cite this article: Barbara L. Klein & Abdulmassih S. Iskandrian (1979) Coarctation of the aorta, Postgraduate Medicine, 66:2, 159-163, DOI: 10.1080/00325481.1979.11715229 To link to this article: http://dx.doi.org/10.1080/00325481.1979.11715229
Published online: 07 Jul 2016.
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Date: 19 August 2017, At: 03:21
HYPOTHETICAL CASE Self-assessment
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Coarctation of the aorta Coarctation of the aorta may cause no symptoms and may be discovered only after hypertension is detected unexpectedly on routine examination. Diagnosis may be made from clinicat fiitdings and results of roentgenography, but additional studies are necessary to define associated cardiac anomalies and the extent of collateral vessel development.
Barbara L. Klein, MD Abdulmassih S. Iskandrian, MD
A 29-year-o/d man who does heavy tabor cornes to the office complaining of frequent occipital headaches during the preceding six months. His general health has been excellent, and his medical history is unremarkable except for a heart murmur which has been present since childhood. At physical examination, his blood pressure is 185/90 mm Hg and a soft systolic murmur is heard along the left sternal border. Aortic pulsation is visible in the suprasternal notch. Blood pressure in the legs is 110/90 mm Hg, and the femoral pulses are weak and delayed compared with the brachial pulses. Results of neurologie examination are unremarkable. Roentgenography of the chest shows mild cardiomega/y and dilation of the aortic root. Notching of the lower margins of the ribs is apparent posteriorly (figure 1). The patient is hospitalized. Question: What is the likely diagnosis? 1. Coarctation of the aorta 2. Pseudocoarctation of the aorta 3. Aortic stenosis
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
4. Dissecting aneurysm of the aorta Answer: 1 1. Aortic coarctation 1s a relatively common entity, compnsmg about 6.5% of ali congenital cardiac anomalies. It is twice as common in males as in females. Symptoms can be minimal, with diagnosis being made only after suspicion is raised by the discovery of high blood pressure on routine examination.
,--'-----· ,_________ 1 1 1 1 1
1 1 1 1 ..1
One of a group of case reports incorporatlng a management seH-test
2. Pseudocoarctation is a rare entity which may be confused with coarctation of the aorta. It is thought to be a kink at the leve! of the aortic knob; however, pressure does not differ above and below the kink. The roentgenographic picture may suggest a mass lesion in the region of the aortic arch. Because blood flow through the aorta is not diminished, no rib notching occurs and peripheral pulses are normal.
Diagnosis is made clinically. 3. The quality of the murmur, the presence of a normal second heart sound, and the disparity in blood pressure between the upper and lower extremities are against the diagnosis of aortic stenosis. 4. The clinical picture and the roentgenographic findings are against the diagnosis of aortic dissection. Question: What causes aortic coarctation? Answer: Formation is related to abnormal development of the fourth left aortic arch, but the etiology of the defect is not weil understood. Ovarian agenesis, or Turner's syndrome, resulting from a chromosomal abnormality is frequently associated with aortic coarctation. Question: What is the significance of notching of the lower rib margins? Answer: Notching of the lower rib margins posteriorly is related to development of dilated and tortuous intercostal arterial channels. Development of collateral vessels is an important feature of coarctation of the aorta, as is enlargement of normal arterial anastomoses above and below the area of constriction. When the constriction is particularly tight, continued 159
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HYPOTHI!TICAL CASI! CONTINUED
the distal aorta receives most of its blood flow through collateral channels from the subclavian and intercostal arteries. High flow through the intercostal arteries causes them to press against rib surfaces, providing the characteristic pattern on x-ray film. If either subclavian artery arises below the area of constriction, rib notching is unilateral and contralateral.
Question: What is the difference between "infantile" and "adult" coarctation of the aorta? Answer: The terms "infantile" and "adult" refer primarily to the location of the aortic constriction rather than to the age of the patient. Infantile coarctation is proximal to the ductus arteriosus and can occur after adolescence, while adult coarctation is distal to the ductus arteriosus and can occur in infancy. The infantile form commonly is accompanied by patent ductus arteriosus and the adult form by bicuspid aortic valve configuration.
sociated anomalies and in part to failure to feed properly. 2. Beyond childhood, patients with aortic coarctation tend to have minimal symptoms until hypertension becomes manifest, usually in young adulthood. 3. Deterioration of the arterial vascular bed in advanced hypertension produces aneurysm, aortic dissection, and cerebrovascular accidents. 4. Few patients with aortic coarctation survive beyond the fifth decade of !ife, even with surgery. In untreated patients, mean age at death is 35 years. Causes of death include congestive heart failure, ruptured aortic aneurysm, cerebrovascular accident, coronary heart disease, and rarely, gross valvular incompetence following bacterial endocarditis.
Question: Which of the following is or are often associated with adult coarctation of the aorta? 1. Claudication 2. Heart failure
Question: Which of the following is or are part of the natural history of aortic coarctation? 1. High mortality in infancy 2. Development of hypertension in adulthood 3. Deterioration of arterial vascular bed 4. Dea th by fifth decade of !ife Answer: 1, 2, 3, 4 1. Mortality is high in infants with coarctation, owing in part to as-
160
Barbara L. Klein Dr Klein was a fellow in cardiology, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, Philadelphia. She is now in priva te practice of cardiology in Las Vegas.
Abdulmassih S. lskandrian Dr Iskandrian is associate professor of cardiology, Hahnemann Medical College and Hospital. He is a staff member of the cardiac catheterization laboratories and head of the nuclear cardiology division, William Likoff Cardiovascijlar Institute.
3. Headache 4. Nosebleed Answer: 3, 4 1. Leg discomfort on exertion occurs only if collateral flow to the lower extremities is poorly developed. 2. Heart failure is common in infants with aortic coarctation but not in adults. 3, 4. Adults with symptomatic coarctation of the aorta have hypertension-related complaints, such as headache and nosebleed.
Question: What is the definitive diagnostic procedure? 1. Roentgenography 2. Cardiac catheterization and aortography 3. Electrocardiography 4. Echocardiography Answer: 2 1. Roentgenography of the chest may suggest the diagnosis and may show notching of the ribs and dilatation of the ascending aorta. It, however, does not provide information on the site, extent, or severity of the coarctation or on the associated cardiac diseases. 2. Aortography delineates the location and length of the constriction (figure 2a). It also defines any associated congenital anomalies and indicates the degree of collateral vesse! development (figure 2b). It allows accurate assessment of aortic valve configuration. Intracardiac catheters placed above and below the area of con-
VOL66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
striction allow measurement of the pressure gradient across the coarctation (figure 3). Aortic insufficiency often is associated with aortic coarctation, and the degree of aortic regurgitation can be weil defined by visualization of the aortic root. 3. In both children and adults with aortic coarctation, the ECG can be normal. In adults, however, it occasionally suggests left ventricular hypertrophy, especially in the presence of long-standing hypertension or aortic valve disease (figure 4). 4. Echocardiography is of limited value in the diagnosis, although it may provide valuable information on the degree of left ventricular hypertrophy, left ventricular function, and associated diseases.
Cardiac catheterization is done on the 14th day of hospitalization. Aortography shows severe coarcta-
2. Dissecting aneurysm of the aorta 3. Subacute bacterial endocarditis 4. Aortic valvular stenosis
Answer: 3, 4 Figure 1. Chest x-ray film in posteroanterior projection shows bilateral notching of lower margins of ribs. Figure 2. Aortograms from patient with aortic coarctation. a. Discrete, localized narrowing of aorta. b. Dilated internai mammary artery and other collateral vessels.
tion of the aorta and bicuspid configuration of the aortic valve. Left ventricular function is normal.
Question: To which of the following conditions or abnormalities is a bicuspid aortic valve particularly vulnerable? 1. Rupture of aneurysm of sinus ofValsalva
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
1. Aneurysms of the sinus of Valsalva are generally of congenital origin and usually involve the right or the posterior coronary cusp, but they are unrelated to the presence of a bicuspid valve. 2. Although dissecting aneurysm of the aorta may be seen in patients with a bicuspid aortic valve, it is generally related to the associated coarctation of the aorta or hypertension, or both, and not to the bicuspid valve per se. 3. Bacterial endocarditis may attack the area of coarctation or the aortic valve, the area of poststenotic dilatation, or the bicuspid aortic valve. 4. The bicuspid aortic valve is subject to development of hemodycontinued
161
HYPOTHI:TICAL CASB CONTINUED
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namically significant aortic stenosis or insufficiency in a certain proportion of such patients after variable ti me. Turbulence of flow, microthrombi, wear and tear, and other factors are postulated to be the responsible mechanisms.
Figure 3. Aortic pressure recordings from patient with aortic coarctation, obtained simultaneously from intracardiac catheters placed above (Asc ao) and below (Des ao) leve) of coarctation. Latter shows lower pressure and delayed upstroke. Figure 4. ECG from patient with aortic coarctation shows left ventricular hypertrophy.
162
Question: What is the correct treatment for aortic coarctation? 1. Surgery 2. Drug therapy to control hypertension 3. Prophylaxis against endocarditis 4. Digitalis Answer: 1 1. Surgical intervention for aortic coarctation bas been carried out successfully since 1945, and over the years, refinements in surgical technique have markedly reduced operative mortality. The area of coarctation is resected and an end-to-end anastomosis is done. If the constricted segment is elongated, a synthetic fiber graft is inserted to bridge the gap. 2. The hypertension associated with aortic coarctation may be resistant to drug therapy. 3. Prophylaxis against endocarditis may still be required even after surgical treatment in patients with a bicuspid aortic valve, but it is only part of a more appropriate therapeutic approach. 4. Digitalis is not indicated in the absence of congestive heart failure. Moreover, such patients should be treated early with surgery before heart failure supervenes.
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
Question: How does surgery affect !ife expectancy? Answer: Surgical correction of coarctation often does not significantly affect !ife expectancy. Sorne studies have indicated that !ife expectancy after corrective surgery is similar to !ife expectancy in the era preceding surgical intervention. Early death following surgery h!ls been attrib~ted to the cardiovascular defects and cerebrovascular lesions associated with aortic coarctation.
presum~bly
because of irreversible arteriosclerotic changes.
Question: Which of the following may be complications of surgery? 1. Paradoxical hypertension 2. Paraplegia 3. Claudication 4. Accelerated coronary heart disease Answer: 1, 2, 3, 4 1. Paradoxical increase in blood pressure immediately after surgery for aortic coarctation is associated with elevated sympathetic disQuestion; When should surgery ide- charge, as evidenced by increased urinary excretion of catecholally be done? amines. The hypertension is wide1. Before the age of 5 years spread and is not limited to the ves2. At age 6 to 8 years sels originating above the coarcta3. At age 10 to 12 years tion. Mesenteric vessels may be 4. In adulthood involved, resulting in bowel infarcAnswer: 2 tion, a serious but fortunately un1. Surgery is best done as early as common complication. Careful atpossible, but until a child is about 6 years old the aorta enlarges substan- tention to blood pressure recordings tially. Growth of an anastomosis after surgery and administration of done before 6 years of age would an antihypertensive agent when indicated usually result in complete probably lag behind aortic growth. 2. At age 6 to 8 years the dimen- recovery. 2. Paraplegia is a recognized sions of the aorta reach the approximate dimensions of the adult aorta. complication of coarctation resection, occurring in about 0.4% of This is the ideal age for surgery. cases. lt may be related to compro3. See answers 1 and 2. 4. Aortic tissue becomes Jess elas- mise of the blood supply to the spinal tic with age. If aortic coarctation is cord during aortic cross-clamping. 3. Many young patients experileft untreated unti1 adulthood, calcifie and arteriosclerotic changes ence claudication and limited exermay be evident and may increase cise tolerance after surgery, related operative mortality. Recent evi- to residual persistent pressure gradidence has suggested that hyperten- ent between arms and legs. More sion may become fixed or irreversi- extensive excision at the site of the ble after the second decade of !ife, anastomosis usually eliminates the
VOL 66/NO 2/ AUGUST 1979/POSTGRADUATE MEDICINE
gradient. 4. Sorne investigations have suggested that premature degenerative changes occur in the coronary arteries of patients with aortic coarctation subjected to surgery after the age of 18 years.
Successful aortic anastomosis is performed on the fourth day after catheterization. Two weeks after surgery the blood pressure is 124/84 mm Hg. The femoral pulses show rapid rise and no significant delay in relation to the brachial pulses. The patient is discharged. Series coordinator for hypothetical cases referable to cardiovascular disease: Bernard L. Segal, MD, professor of medicine and director, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, Philadelphia. Address reprint requests to Abdulmassih S. lskandrian, MD, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, 230 N Broad St, Philadelphia, PA 19102. Bibliography Ca111pbell M, Bayliss JH: The course and prognosis of coarctation of the aorta. Br Heart J 18:475-495, 1956 Lindesmith GG et al: Coarctation of the thoracic aorta. Ann Thorac Cardiovasc Surg Il :482-497, 1971 Sealy WC et al: Paradoxical hypertension following resection of coarctation of aorta. Surgery 42:135-147, 1957 Silber EN, Katz LN: Heart Disease. New York. Macmillan Publishing Co lnc, 1975 Simon AB, Zloto AE: Coarctation of the aorta: Longitudinal assessment of operated patients. Circulation 50:456-464, 1974
163
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Coarctation of the aorta Barbara L. Klein & Abdulmassih S. Iskandrian To cite this article: Barbara L. Klein & Abdulmassih S. Iskandrian (1979) Coarctation of the aorta, Postgraduate Medicine, 66:2, 159-163, DOI: 10.1080/00325481.1979.11715229 To link to this article: http://dx.doi.org/10.1080/00325481.1979.11715229
Published online: 07 Jul 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ipgm20 Download by: [Australian Catholic University]
Date: 19 August 2017, At: 03:21
HYPOTHETICAL CASE Self-assessment
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
Coarctation of the aorta Coarctation of the aorta may cause no symptoms and may be discovered only after hypertension is detected unexpectedly on routine examination. Diagnosis may be made from clinicat fiitdings and results of roentgenography, but additional studies are necessary to define associated cardiac anomalies and the extent of collateral vessel development.
Barbara L. Klein, MD Abdulmassih S. Iskandrian, MD
A 29-year-o/d man who does heavy tabor cornes to the office complaining of frequent occipital headaches during the preceding six months. His general health has been excellent, and his medical history is unremarkable except for a heart murmur which has been present since childhood. At physical examination, his blood pressure is 185/90 mm Hg and a soft systolic murmur is heard along the left sternal border. Aortic pulsation is visible in the suprasternal notch. Blood pressure in the legs is 110/90 mm Hg, and the femoral pulses are weak and delayed compared with the brachial pulses. Results of neurologie examination are unremarkable. Roentgenography of the chest shows mild cardiomega/y and dilation of the aortic root. Notching of the lower margins of the ribs is apparent posteriorly (figure 1). The patient is hospitalized. Question: What is the likely diagnosis? 1. Coarctation of the aorta 2. Pseudocoarctation of the aorta 3. Aortic stenosis
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
4. Dissecting aneurysm of the aorta Answer: 1 1. Aortic coarctation 1s a relatively common entity, compnsmg about 6.5% of ali congenital cardiac anomalies. It is twice as common in males as in females. Symptoms can be minimal, with diagnosis being made only after suspicion is raised by the discovery of high blood pressure on routine examination.
,--'-----· ,_________ 1 1 1 1 1
1 1 1 1 ..1
One of a group of case reports incorporatlng a management seH-test
2. Pseudocoarctation is a rare entity which may be confused with coarctation of the aorta. It is thought to be a kink at the leve! of the aortic knob; however, pressure does not differ above and below the kink. The roentgenographic picture may suggest a mass lesion in the region of the aortic arch. Because blood flow through the aorta is not diminished, no rib notching occurs and peripheral pulses are normal.
Diagnosis is made clinically. 3. The quality of the murmur, the presence of a normal second heart sound, and the disparity in blood pressure between the upper and lower extremities are against the diagnosis of aortic stenosis. 4. The clinical picture and the roentgenographic findings are against the diagnosis of aortic dissection. Question: What causes aortic coarctation? Answer: Formation is related to abnormal development of the fourth left aortic arch, but the etiology of the defect is not weil understood. Ovarian agenesis, or Turner's syndrome, resulting from a chromosomal abnormality is frequently associated with aortic coarctation. Question: What is the significance of notching of the lower rib margins? Answer: Notching of the lower rib margins posteriorly is related to development of dilated and tortuous intercostal arterial channels. Development of collateral vessels is an important feature of coarctation of the aorta, as is enlargement of normal arterial anastomoses above and below the area of constriction. When the constriction is particularly tight, continued 159
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
HYPOTHI!TICAL CASI! CONTINUED
the distal aorta receives most of its blood flow through collateral channels from the subclavian and intercostal arteries. High flow through the intercostal arteries causes them to press against rib surfaces, providing the characteristic pattern on x-ray film. If either subclavian artery arises below the area of constriction, rib notching is unilateral and contralateral.
Question: What is the difference between "infantile" and "adult" coarctation of the aorta? Answer: The terms "infantile" and "adult" refer primarily to the location of the aortic constriction rather than to the age of the patient. Infantile coarctation is proximal to the ductus arteriosus and can occur after adolescence, while adult coarctation is distal to the ductus arteriosus and can occur in infancy. The infantile form commonly is accompanied by patent ductus arteriosus and the adult form by bicuspid aortic valve configuration.
sociated anomalies and in part to failure to feed properly. 2. Beyond childhood, patients with aortic coarctation tend to have minimal symptoms until hypertension becomes manifest, usually in young adulthood. 3. Deterioration of the arterial vascular bed in advanced hypertension produces aneurysm, aortic dissection, and cerebrovascular accidents. 4. Few patients with aortic coarctation survive beyond the fifth decade of !ife, even with surgery. In untreated patients, mean age at death is 35 years. Causes of death include congestive heart failure, ruptured aortic aneurysm, cerebrovascular accident, coronary heart disease, and rarely, gross valvular incompetence following bacterial endocarditis.
Question: Which of the following is or are often associated with adult coarctation of the aorta? 1. Claudication 2. Heart failure
Question: Which of the following is or are part of the natural history of aortic coarctation? 1. High mortality in infancy 2. Development of hypertension in adulthood 3. Deterioration of arterial vascular bed 4. Dea th by fifth decade of !ife Answer: 1, 2, 3, 4 1. Mortality is high in infants with coarctation, owing in part to as-
160
Barbara L. Klein Dr Klein was a fellow in cardiology, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, Philadelphia. She is now in priva te practice of cardiology in Las Vegas.
Abdulmassih S. lskandrian Dr Iskandrian is associate professor of cardiology, Hahnemann Medical College and Hospital. He is a staff member of the cardiac catheterization laboratories and head of the nuclear cardiology division, William Likoff Cardiovascijlar Institute.
3. Headache 4. Nosebleed Answer: 3, 4 1. Leg discomfort on exertion occurs only if collateral flow to the lower extremities is poorly developed. 2. Heart failure is common in infants with aortic coarctation but not in adults. 3, 4. Adults with symptomatic coarctation of the aorta have hypertension-related complaints, such as headache and nosebleed.
Question: What is the definitive diagnostic procedure? 1. Roentgenography 2. Cardiac catheterization and aortography 3. Electrocardiography 4. Echocardiography Answer: 2 1. Roentgenography of the chest may suggest the diagnosis and may show notching of the ribs and dilatation of the ascending aorta. It, however, does not provide information on the site, extent, or severity of the coarctation or on the associated cardiac diseases. 2. Aortography delineates the location and length of the constriction (figure 2a). It also defines any associated congenital anomalies and indicates the degree of collateral vesse! development (figure 2b). It allows accurate assessment of aortic valve configuration. Intracardiac catheters placed above and below the area of con-
VOL66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
striction allow measurement of the pressure gradient across the coarctation (figure 3). Aortic insufficiency often is associated with aortic coarctation, and the degree of aortic regurgitation can be weil defined by visualization of the aortic root. 3. In both children and adults with aortic coarctation, the ECG can be normal. In adults, however, it occasionally suggests left ventricular hypertrophy, especially in the presence of long-standing hypertension or aortic valve disease (figure 4). 4. Echocardiography is of limited value in the diagnosis, although it may provide valuable information on the degree of left ventricular hypertrophy, left ventricular function, and associated diseases.
Cardiac catheterization is done on the 14th day of hospitalization. Aortography shows severe coarcta-
2. Dissecting aneurysm of the aorta 3. Subacute bacterial endocarditis 4. Aortic valvular stenosis
Answer: 3, 4 Figure 1. Chest x-ray film in posteroanterior projection shows bilateral notching of lower margins of ribs. Figure 2. Aortograms from patient with aortic coarctation. a. Discrete, localized narrowing of aorta. b. Dilated internai mammary artery and other collateral vessels.
tion of the aorta and bicuspid configuration of the aortic valve. Left ventricular function is normal.
Question: To which of the following conditions or abnormalities is a bicuspid aortic valve particularly vulnerable? 1. Rupture of aneurysm of sinus ofValsalva
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
1. Aneurysms of the sinus of Valsalva are generally of congenital origin and usually involve the right or the posterior coronary cusp, but they are unrelated to the presence of a bicuspid valve. 2. Although dissecting aneurysm of the aorta may be seen in patients with a bicuspid aortic valve, it is generally related to the associated coarctation of the aorta or hypertension, or both, and not to the bicuspid valve per se. 3. Bacterial endocarditis may attack the area of coarctation or the aortic valve, the area of poststenotic dilatation, or the bicuspid aortic valve. 4. The bicuspid aortic valve is subject to development of hemodycontinued
161
HYPOTHI:TICAL CASB CONTINUED
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
namically significant aortic stenosis or insufficiency in a certain proportion of such patients after variable ti me. Turbulence of flow, microthrombi, wear and tear, and other factors are postulated to be the responsible mechanisms.
Figure 3. Aortic pressure recordings from patient with aortic coarctation, obtained simultaneously from intracardiac catheters placed above (Asc ao) and below (Des ao) leve) of coarctation. Latter shows lower pressure and delayed upstroke. Figure 4. ECG from patient with aortic coarctation shows left ventricular hypertrophy.
162
Question: What is the correct treatment for aortic coarctation? 1. Surgery 2. Drug therapy to control hypertension 3. Prophylaxis against endocarditis 4. Digitalis Answer: 1 1. Surgical intervention for aortic coarctation bas been carried out successfully since 1945, and over the years, refinements in surgical technique have markedly reduced operative mortality. The area of coarctation is resected and an end-to-end anastomosis is done. If the constricted segment is elongated, a synthetic fiber graft is inserted to bridge the gap. 2. The hypertension associated with aortic coarctation may be resistant to drug therapy. 3. Prophylaxis against endocarditis may still be required even after surgical treatment in patients with a bicuspid aortic valve, but it is only part of a more appropriate therapeutic approach. 4. Digitalis is not indicated in the absence of congestive heart failure. Moreover, such patients should be treated early with surgery before heart failure supervenes.
VOL 66/NO 2/AUGUST 1979/POSTGRADUATE MEDICINE
Downloaded by [Australian Catholic University] at 03:21 19 August 2017
Question: How does surgery affect !ife expectancy? Answer: Surgical correction of coarctation often does not significantly affect !ife expectancy. Sorne studies have indicated that !ife expectancy after corrective surgery is similar to !ife expectancy in the era preceding surgical intervention. Early death following surgery h!ls been attrib~ted to the cardiovascular defects and cerebrovascular lesions associated with aortic coarctation.
presum~bly
because of irreversible arteriosclerotic changes.
Question: Which of the following may be complications of surgery? 1. Paradoxical hypertension 2. Paraplegia 3. Claudication 4. Accelerated coronary heart disease Answer: 1, 2, 3, 4 1. Paradoxical increase in blood pressure immediately after surgery for aortic coarctation is associated with elevated sympathetic disQuestion; When should surgery ide- charge, as evidenced by increased urinary excretion of catecholally be done? amines. The hypertension is wide1. Before the age of 5 years spread and is not limited to the ves2. At age 6 to 8 years sels originating above the coarcta3. At age 10 to 12 years tion. Mesenteric vessels may be 4. In adulthood involved, resulting in bowel infarcAnswer: 2 tion, a serious but fortunately un1. Surgery is best done as early as common complication. Careful atpossible, but until a child is about 6 years old the aorta enlarges substan- tention to blood pressure recordings tially. Growth of an anastomosis after surgery and administration of done before 6 years of age would an antihypertensive agent when indicated usually result in complete probably lag behind aortic growth. 2. At age 6 to 8 years the dimen- recovery. 2. Paraplegia is a recognized sions of the aorta reach the approximate dimensions of the adult aorta. complication of coarctation resection, occurring in about 0.4% of This is the ideal age for surgery. cases. lt may be related to compro3. See answers 1 and 2. 4. Aortic tissue becomes Jess elas- mise of the blood supply to the spinal tic with age. If aortic coarctation is cord during aortic cross-clamping. 3. Many young patients experileft untreated unti1 adulthood, calcifie and arteriosclerotic changes ence claudication and limited exermay be evident and may increase cise tolerance after surgery, related operative mortality. Recent evi- to residual persistent pressure gradidence has suggested that hyperten- ent between arms and legs. More sion may become fixed or irreversi- extensive excision at the site of the ble after the second decade of !ife, anastomosis usually eliminates the
VOL 66/NO 2/ AUGUST 1979/POSTGRADUATE MEDICINE
gradient. 4. Sorne investigations have suggested that premature degenerative changes occur in the coronary arteries of patients with aortic coarctation subjected to surgery after the age of 18 years.
Successful aortic anastomosis is performed on the fourth day after catheterization. Two weeks after surgery the blood pressure is 124/84 mm Hg. The femoral pulses show rapid rise and no significant delay in relation to the brachial pulses. The patient is discharged. Series coordinator for hypothetical cases referable to cardiovascular disease: Bernard L. Segal, MD, professor of medicine and director, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, Philadelphia. Address reprint requests to Abdulmassih S. lskandrian, MD, William Likoff Cardiovascular lnstitute, Hahnemann Medical College and Hospital, 230 N Broad St, Philadelphia, PA 19102. Bibliography Ca111pbell M, Bayliss JH: The course and prognosis of coarctation of the aorta. Br Heart J 18:475-495, 1956 Lindesmith GG et al: Coarctation of the thoracic aorta. Ann Thorac Cardiovasc Surg Il :482-497, 1971 Sealy WC et al: Paradoxical hypertension following resection of coarctation of aorta. Surgery 42:135-147, 1957 Silber EN, Katz LN: Heart Disease. New York. Macmillan Publishing Co lnc, 1975 Simon AB, Zloto AE: Coarctation of the aorta: Longitudinal assessment of operated patients. Circulation 50:456-464, 1974
163
