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Long-term Severe Pulmonary Hypertension Associated with Right Atrial Myxoma
Long-term Severe Pulmonary Hypertension Associated with Right Atrial Myxoma* Herman A. lleck jr., M.D ., F.C.C.P.;t Charles M. Gmss. M .D .;t and Jan L. Houghton , M.D .t
An IS-year-old black woman presented with marginally compensated right heart failure, severe pulmonary hypertension, tricuspid incompetence, and right atrial myxoma. Catheterization suggested a substantial reactive component to her P-HTN, especially to nifedipine. Initial management consisted of excision of two right atrial myxomas and tricuspid annuloplasty, and postdischarge management with nifedipine, 30 mg four times daily. Emergency pulmonary thromboendarterectomy was required two weeks later for acute cor pulmonale. It is suggested that concomitant procedures are mandatory in this setting because of the otherwise accelerated adverse pathophysiology of obliterative pulmonary vascular obstructive disease. (Chest 1992; 102:301-03) MCG = Medical College of Georgia; P-TEA = pulmonary thromboendarterectomy; P-HTN pulmonary arterial hypertension; PVOD pulmonary vascular obstructive disease; r-tPA = recombinant tissue plasminogen activator
=
=
T
he association of prolonged severe pulmonary arterial hypertension from embolic sequelae of right atrial myxoma has been infrequently described and has not been reported as having been successfully managed . J We have had the opportunity to manage such a patient with initial excision of the myxoma which necessitated subsequent emergency pulmonary thromboendarterectomy a few weeks later. The unusual association of these two lesions and the difficulties encountered as a result of our initial approach prompt liS to report this ease. CASE REPORT
An 18-year-old hlack woman was initially admitted to the Medical College of Georgia on Feh. Ii, 1990, for cesarean section delivery of a 28-week breech pregnancy complicated by preeclampsia and fetal distress. She had a past history of hypertension, recurrent pyelonephritis, and nephrotic syndrome since 198i, and a history of chronic "asthma" treated intermittently with bronchodilators since age ten . She was discharged from MCG on March 3, 1990, but was readmitted to her local hospital March Ii, 1990, with left pleu rit ic chest pain, hemoptysis, and exacerbation of her dyspnea . Perfusion lung scan and isolated left pulmonary arteriogram documented a left lower lobe pulmonary embolus for which she was subsequently treated with ten days of intravenous heparin and conversion to warfarin. Repeat perfusion lung scan prior to discharge revealed no change in her left-sided filling defect and suggested then , and in retrospect, the presence of a right lower lobe defect. On May 4, 1990, she was readmitted to MCG with recurrent pleuritic pain, progressive dyspnea, and ECG evidence of right ventricular strain. Pertinent laboratory findings were : Po" 83; Pco, 39; pH , 7.44; 0, saturation, 94 percent; prothrombin time , 14.8 s; ·From tilt' Sections of Thoracic and Cardiovascular Surgery and Adult Cardiology, The Medical College of Georgia, Augusta . t Assistant Professor of Surgery/Cardiothoracic Surgery. tAssociate Professor of Medicine/Cardiology. Reprint requests: Dr. Heck. Section of Thoracic-Cardiac Surgery . Medica! College of Georgia, Augusta .30912-4040
FIGURE 1. Preoperative pulmonary arteriogram demonstrating complete occlusion of right lower lobe pulmonary artery with distal eollateralization, and subtotal occlusion ofleft lower lobe pulmonary artery.
partial thromboplastin time, 66.2 s. Over the ensuing three weeks , during which time she received anticoagulation with intravenous heparin, she underwent an extensive work-up as follows: echocardiogram revealed dilated right-sided chambers, moderate tricuspid regurgitation, and a right atrial mass; repeat perfusion lung scan confirmed bilateral lower lobe defects similar to previous studies and consistent with pulmonary embolus; pulmonary arteriograms confirmed bilateral lower lohe occlusions (Fig I); pulmonary function test s revealed an FEV, of 1.66 L (68 percent) and an FVC of 1.93 L (64 percent) consistent with mild restrictive ventilatory impairment; exercise blood gas values showed significant desaturation, with resting Po, of 84 mm Hg (95 percent) and exercise Po, of 58 mm Hg (90.2 percent); lower extremity venograms and IVC and SVC venograms were normal, as were upper and lower extremity Doppler studies; coaguation work-up was negative for Creactive protein or antithrombin 3 deficiency. Cardiac catheterization performed on May IS, 1990, substantiated severe P-JITN and severe elevation of pulmonary vascular resistance (Table I). Moder ate improvement of these parameters occurred with 100 percent oxygen and intravenous nitroglycerin and marked improvement occurred with sublingual nifedipine. On May 23, 1990, surgery for excision of her atrial myxoma without concomitant P-TEA was undertaken with removal of two separate, pedunculated myxomas which were attached to the right atrial wall. Concomitant tricuspid annuloplasty was accomplished, and her operative and postoperative course W,L~ essentially uncornplicated . She was discharged ten days later receiving oral theophylline, furosemide, digoxin, and nifedipinc . the latter in a dose of30 mg qid . She was readmitted June 18, 1990, with progressive dyspnea, orthopnea, and arterial desaturation (Po" 48 mm Hg, Pco, 26 mm JIg ; pH , i.45; 0, saturation, 79 percent). The chest x-ray film was unremarkable , but the echocardiogram revealed severe right ventr icular enlargement with global hypokinesis and severe P-HTN . She was started on oxygen and intravenous heparin. On June 20, 1990, she developed acute hemodynam ic collapse associated with right atrial thrombosis which was treated successfully with intravenous r-tPA, intraaortic balloon support, mechanical ventilation, and dopamine. Over the ensuing 48 h , she remained unstable with CHEST I 102 I 1 I JULY, 1992
301
Table I-Catheterization Hemodynamics· Pre Op (5-15-90)*
A... rum Ill.: I'A. 111m Ill.: )'C\\·. "UII Ill.:
149/1lil (110) 74/29 10/13
1\\: mm Ill.:
II" . 111m Ill.: )'\'11 . d Y"I'~' S"( 'm ' S\'II . dym-vs-cm '0
( ;().I/mi"
!W17
(46)
(12)
1.511 .'3 (12) 36.'3 104.'5 7..'5
llKI% 0,
SLTNG
SLN
154190 (113) .'59/19 (36)
160/102 (110)
139191 (107) 44/11 (22)
53/19
(31)
Post Op (11-16-90) Baseline 156183 (110) 44/11 (27) 18125 (16) 53/13
256 1077
203 1173
107 1013
lUll 116
(7)
1074
7.6
'1'\'11. pulm.. nary vascular re-sistance: SVR. systemic vascular resistance: SLTNG, sublingual nitroglycerine, SLN, sublingual nifedipine.
I'rtlJ.(rt'ssiv,· n 'nal Iailur« and with myocl..nit' seiznn- activity, On )111'" 22. HJ90. sl.., was returned t.. Ihl' "p, ·ralinl.: room where hilatr -ral I'-TEA was perforuu-d utilizin~ tw.. separate periods of «in -ulat.. r~ arn-st of 3.'3 and 20 min. resp,-,: tin,ly. Removal of a Iihrous dluphragm oc clnding the right lower loh.- pulmonary art er y malt-rial ocelud in~ the left and a proximal 11m' of friable. or~anized Imn' r 101... pulmonarv arlt'ry was accomplished . j'l;o fresh thromhus orilic,'s were unobstruct ed . was " ',·n. and ..tlu-r lohar and se~mental Suhsr -qru-nt h istop atll l o~it' stud ies n-veuk-d this to he consistent wilh chroni« ,'mholi(' myxomu (F i~ 2). 11"r p..stop,·rativ,' ('our'" was complicated hy transient renal ",ilun' n''Iuirin~ It'mporary dialysis. prtlJon~l'd ventilatorv support
FIC:l'IIE 2, IA'ft pulmonarv artery specimen eonfirmuu; the myx..maltl1ls hist.. lo~i( ' appearance similar 10 that seen in sections from II..· pn 'vio"sly n'mo\,,'d right ntrial myxomas (ori~inal magnlficntion
x UKI),
and pneumonia , and a resolving basal ganglia deficit manifested as choreiform extremity movements. After ten weeks of hospitalization and rehabilitation . she was discharged on a regimen of warfarin and nifedipine , Follow-up cardiac catheterization on November 15, 1990, confirmed only mildly elevated pulmonary artery pressures and resistances (Table 1). Marked improvement in perfusion of both lower lobes was confirmed by repeat perfusion lung scan and pulmonary angiogram confirmed bilateral patenc y of lower lobe pulmonary arteries (Fig 3). When last seen nine months following her P-TEA, she exhibited minimal intention-tremor of her upper extremities . DISCUSSION
The treatment of isolated, chronic, thromboembolic P-HTN by P-TEA has now been well established ..-sHowever, few data are available as to the natural history of recurrent small tumor emboli from right atrial myxoma vis-a-vis the development of substantial P-HTN . much less treatment options when these two problems coexist. 1•• ,7 Our decision to perform initial myxoma excision without concomitant P-TEA was based on hemodynamic data which suggested a reactive pulmonary vascular bed and which did not suggest decompensated right heart failure . We felt that postoperative treatment with appropriately high-dose calcium-channel blocker therapy as suggested-w previously would allow a less risky procedure to be performed and that hemodynamic evaluation of the need for P-TEA could later be made with more certainty. Her subsequent episode of acute cor pulmonale emphasizes the danger in our initial approach. Therefore, it is suggested that a combined surgical approach as initial mangement is mandatory in the setting of severe secondary P-HTN in association with right atrial myxoma and can be expected to be associated with acceptably low mortality, as reported by others. II REFERENCES
Heath D, Mackinnon J. Pulmonary hypertension due to myxoma of the right atrium. Am Heart J 1964; 68 :227-35 2 Ch itwood WR, Lyerly HK, Sabiston DC. Surgical management of chronic pulmonary embolism . Ann Surg 1985; 201 :11-26 3 Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 1990; 81:1735-43
F"" 'IIF :l, Post"p"rativ,' pulmonary cineangiogrum demonstrattng ....mpk-te rr-storation of flow t.. IM,th lower lohe pulmonary arteries.
302
4 Daily PO. Surgical management of chronic pulmonary embolism. In: Bergan J], Yao JST, eds. Venousdisorders. Philadelphia: WB Saunders Co, 1991:531-41 5 Daily PO, Demhitsky wa Peterson KL, Moser KM. Modifications of techniques and early results of pulmonary thromboendarterectomy for chronic pulmonary embolism . J Thorne CarPulmonary Hypertension and Right Atrial Myxoma (Heck. Gross. Houghton)
I
diovasc Surg 1987; 93:221-33
6 Keller H, Stegaru B, Buss 7 8
9
10
11
J,
Genth K, Heene D. Pulmonary tumor embolism and right atrial myxoma detected by twodimensional echocardiography. Am Heart J 1985; 110:881-84 Abdur RT, Gross H, Siegelman SS. Right atrial myxoma and pulmonary hypertension. NY State J Med 1970; 70:2996-3000 Riedel M, Stanek ~ Widimsky J, Prerovsky I. Longterm followup of patients with pulmonary thromboembolism: late prognosis and evolution of hemodynamic and respiratory data. Chest 1982; 81:151-58 Rich S, Brundage BH, Levy PS. The effect of vasodilator therapy on the clinical outcome of patients with primary pulmonary hypertension. Circulation 1985; 71:1191-96 Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation 1987; 76:135-41 Daily PO, Dembitsky W~ Iversen S, Moser KM, Auger W Current early results of pulmonary thromboendarterectomy for chronic pulmonary embolism. Eur J Cardiothoracic Surg 1990; 4:117-23
J
In this report, we present a case where a patient needed 75 mg of captopril every 3 h for the control of his blood pressure. CASE REpORT
A 68-yr-old white man had a history of hypertension for over 15 years, and his BP had ranged from 200 to 218 mm Hg systolic and from 100 to 130 mm Hg diastolic. His peripheral venous plasma renin activity (PRA) was 10.4 nwmllh, and a renal scan showed a symmetric uptake and washout pro6le, with no asymmetry in renal size. Initially, the patient required 75 mg of captopril three times daily before meals and 50 mg of hydrochlorothiazide daily to lower his BP to 160180 mm Hg. His caring wife, who was trained to record B~ measured it for several days before and 2 to 3 h after each dose of captopril following any change in dosing schedule. After 6 rno, the BP rose again and fluctuated between 200190and 2101100 mm Hg. Review of the BP chart maintained by the wife indicated a rise only before the next dose was due. No cause for secondary resistance to captopril, such as a change in diet, salt intake, or physical activity, could be detected. There had been no problem with compliance, as indicated by pill counts. Instead of changing to another class of antihypertensive agent, as is customarily done in therapeutic failure, we elected to administer doses of captopril every 6 h, and the BP stabilized in the range of 156'70 to 170180 mm Hg. We had been encouraged to do so because of the lack of side effects from captopril in this patient. The BP went out of control during the next year, when a renal scan revealed markedly decreased size of and flow in the left kidney, and a renal arteriogram confirmed left renal artery occlusion. The left renal vein PRA was 130 nglml/h (right renal vein PRA, 70 nw ml/h, and inferior vena cava PRA, 71 ng/ml/h), As revascularization was not indicated for the severely shrunken left kidney, the patient was offered left nephrectomy, which he refused. Therapy with nifedipine was then added, and the BP remained under control for about 6 mo: however, the BP went out of control once again before each dose of captopril every 6 h, which was then increased to every 4 h, te, to 450 mglday (the maximum recommended dosage per the package insert). This lowered the BP only partially, into the range of 180180to 200190mm Hg. The dosing interval was then decreased to every 3 h, with careful monitoring of subjective and objective
Suman \Urdan, M.D.; Sa1ctipada Mookherjee, M.D.; and
Harold Smulyan, M.D.
The renovascular hypertension of an elderly patient, which was controlled initially with standard-dose therapy with captopril, later responded only to 75 mg given every 3 h without side effects. Changeover to another class of antihypertensives, as is recommmended in recalcitrant cases, was not needed. Convenient maintenance therapy with lisinopril (10 mg twice daily) has kept blood pressure under control for a year. (Chelt 1992; 102:303-04) *From the Sections of Cardiology (Drs. Vardan, Mookberjee, and Smulyan) and General Medicine (Dr. Vardan), Department of Medicine, Veterans Administration Medical and Health Science Centers, State University of New York, Syracuse. Reprint requests:Dr. \flrdan, VA MedicalCenter, 800 Irving Avenue,
Syracuse 13210-2799
200
CD
I
the treatment of hypertension, the usual recommended I"ndose of captopril is 25 to 50 mg taken at 8-h intervals.
Captoprll Administered Every Three Hours in Difficult-to-Control Hypertension
3 Hourly
PM = plasma renin activity
3 Hotxly
6 Hourly
4 Hourly
0-0 Systolic . - - . Diastole
J:
~~
~8 WJ: a: O
150~
SF a: ea:.
100
~~ WW
.. 0
o~
9 m
50
O~"" " " ' ' ' ' ' ' ' 2' ' ' . . -5I' ' ' ' .8. -I' ' 112 ' ' ' ' ' ' ' '_' -5' ' ' ' ' 8' -' ' ' "112 " ", ,' ' ' ' ' ' ' 5' ' ' ' ' 811 '_ 2 5
9 1 5 9 1 5 9 1 5 9 1
-PM- -NI- -PM- -AM-
~
-AM-
- PM-
TIME
10 4
-AM-
10 4
-PM-
10 4
-NIt-
10
-PM-
7 101471015 ~-PM-
§
-AM-
FIGURE 1. Frequency of captopril administration and BP response in hypertensive patient. CHEST I 102 I 1 I JUL~
1992
303
An IS-year-old black woman presented with marginally compensated right heart failure, severe pulmonary hypertension, tricuspid incompetence, and right atrial myxoma. Catheterization suggested a substantial reactive component to her P-HTN, especially to nifedipine. Initial management consisted of excision of two right atrial myxomas and tricuspid annuloplasty, and postdischarge management with nifedipine, 30 mg four times daily. Emergency pulmonary thromboendarterectomy was required two weeks later for acute cor pulmonale. It is suggested that concomitant procedures are mandatory in this setting because of the otherwise accelerated adverse pathophysiology of obliterative pulmonary vascular obstructive disease. (Chest 1992; 102:301-03) MCG = Medical College of Georgia; P-TEA = pulmonary thromboendarterectomy; P-HTN pulmonary arterial hypertension; PVOD pulmonary vascular obstructive disease; r-tPA = recombinant tissue plasminogen activator
=
=
T
he association of prolonged severe pulmonary arterial hypertension from embolic sequelae of right atrial myxoma has been infrequently described and has not been reported as having been successfully managed . J We have had the opportunity to manage such a patient with initial excision of the myxoma which necessitated subsequent emergency pulmonary thromboendarterectomy a few weeks later. The unusual association of these two lesions and the difficulties encountered as a result of our initial approach prompt liS to report this ease. CASE REPORT
An 18-year-old hlack woman was initially admitted to the Medical College of Georgia on Feh. Ii, 1990, for cesarean section delivery of a 28-week breech pregnancy complicated by preeclampsia and fetal distress. She had a past history of hypertension, recurrent pyelonephritis, and nephrotic syndrome since 198i, and a history of chronic "asthma" treated intermittently with bronchodilators since age ten . She was discharged from MCG on March 3, 1990, but was readmitted to her local hospital March Ii, 1990, with left pleu rit ic chest pain, hemoptysis, and exacerbation of her dyspnea . Perfusion lung scan and isolated left pulmonary arteriogram documented a left lower lobe pulmonary embolus for which she was subsequently treated with ten days of intravenous heparin and conversion to warfarin. Repeat perfusion lung scan prior to discharge revealed no change in her left-sided filling defect and suggested then , and in retrospect, the presence of a right lower lobe defect. On May 4, 1990, she was readmitted to MCG with recurrent pleuritic pain, progressive dyspnea, and ECG evidence of right ventricular strain. Pertinent laboratory findings were : Po" 83; Pco, 39; pH , 7.44; 0, saturation, 94 percent; prothrombin time , 14.8 s; ·From tilt' Sections of Thoracic and Cardiovascular Surgery and Adult Cardiology, The Medical College of Georgia, Augusta . t Assistant Professor of Surgery/Cardiothoracic Surgery. tAssociate Professor of Medicine/Cardiology. Reprint requests: Dr. Heck. Section of Thoracic-Cardiac Surgery . Medica! College of Georgia, Augusta .30912-4040
FIGURE 1. Preoperative pulmonary arteriogram demonstrating complete occlusion of right lower lobe pulmonary artery with distal eollateralization, and subtotal occlusion ofleft lower lobe pulmonary artery.
partial thromboplastin time, 66.2 s. Over the ensuing three weeks , during which time she received anticoagulation with intravenous heparin, she underwent an extensive work-up as follows: echocardiogram revealed dilated right-sided chambers, moderate tricuspid regurgitation, and a right atrial mass; repeat perfusion lung scan confirmed bilateral lower lobe defects similar to previous studies and consistent with pulmonary embolus; pulmonary arteriograms confirmed bilateral lower lohe occlusions (Fig I); pulmonary function test s revealed an FEV, of 1.66 L (68 percent) and an FVC of 1.93 L (64 percent) consistent with mild restrictive ventilatory impairment; exercise blood gas values showed significant desaturation, with resting Po, of 84 mm Hg (95 percent) and exercise Po, of 58 mm Hg (90.2 percent); lower extremity venograms and IVC and SVC venograms were normal, as were upper and lower extremity Doppler studies; coaguation work-up was negative for Creactive protein or antithrombin 3 deficiency. Cardiac catheterization performed on May IS, 1990, substantiated severe P-JITN and severe elevation of pulmonary vascular resistance (Table I). Moder ate improvement of these parameters occurred with 100 percent oxygen and intravenous nitroglycerin and marked improvement occurred with sublingual nifedipine. On May 23, 1990, surgery for excision of her atrial myxoma without concomitant P-TEA was undertaken with removal of two separate, pedunculated myxomas which were attached to the right atrial wall. Concomitant tricuspid annuloplasty was accomplished, and her operative and postoperative course W,L~ essentially uncornplicated . She was discharged ten days later receiving oral theophylline, furosemide, digoxin, and nifedipinc . the latter in a dose of30 mg qid . She was readmitted June 18, 1990, with progressive dyspnea, orthopnea, and arterial desaturation (Po" 48 mm Hg, Pco, 26 mm JIg ; pH , i.45; 0, saturation, 79 percent). The chest x-ray film was unremarkable , but the echocardiogram revealed severe right ventr icular enlargement with global hypokinesis and severe P-HTN . She was started on oxygen and intravenous heparin. On June 20, 1990, she developed acute hemodynam ic collapse associated with right atrial thrombosis which was treated successfully with intravenous r-tPA, intraaortic balloon support, mechanical ventilation, and dopamine. Over the ensuing 48 h , she remained unstable with CHEST I 102 I 1 I JULY, 1992
301
Table I-Catheterization Hemodynamics· Pre Op (5-15-90)*
A... rum Ill.: I'A. 111m Ill.: )'C\\·. "UII Ill.:
149/1lil (110) 74/29 10/13
1\\: mm Ill.:
II" . 111m Ill.: )'\'11 . d Y"I'~' S"( 'm ' S\'II . dym-vs-cm '0
( ;().I/mi"
!W17
(46)
(12)
1.511 .'3 (12) 36.'3 104.'5 7..'5
llKI% 0,
SLTNG
SLN
154190 (113) .'59/19 (36)
160/102 (110)
139191 (107) 44/11 (22)
53/19
(31)
Post Op (11-16-90) Baseline 156183 (110) 44/11 (27) 18125 (16) 53/13
256 1077
203 1173
107 1013
lUll 116
(7)
1074
7.6
'1'\'11. pulm.. nary vascular re-sistance: SVR. systemic vascular resistance: SLTNG, sublingual nitroglycerine, SLN, sublingual nifedipine.
I'rtlJ.(rt'ssiv,· n 'nal Iailur« and with myocl..nit' seiznn- activity, On )111'" 22. HJ90. sl.., was returned t.. Ihl' "p, ·ralinl.: room where hilatr -ral I'-TEA was perforuu-d utilizin~ tw.. separate periods of «in -ulat.. r~ arn-st of 3.'3 and 20 min. resp,-,: tin,ly. Removal of a Iihrous dluphragm oc clnding the right lower loh.- pulmonary art er y malt-rial ocelud in~ the left and a proximal 11m' of friable. or~anized Imn' r 101... pulmonarv arlt'ry was accomplished . j'l;o fresh thromhus orilic,'s were unobstruct ed . was " ',·n. and ..tlu-r lohar and se~mental Suhsr -qru-nt h istop atll l o~it' stud ies n-veuk-d this to he consistent wilh chroni« ,'mholi(' myxomu (F i~ 2). 11"r p..stop,·rativ,' ('our'" was complicated hy transient renal ",ilun' n''Iuirin~ It'mporary dialysis. prtlJon~l'd ventilatorv support
FIC:l'IIE 2, IA'ft pulmonarv artery specimen eonfirmuu; the myx..maltl1ls hist.. lo~i( ' appearance similar 10 that seen in sections from II..· pn 'vio"sly n'mo\,,'d right ntrial myxomas (ori~inal magnlficntion
x UKI),
and pneumonia , and a resolving basal ganglia deficit manifested as choreiform extremity movements. After ten weeks of hospitalization and rehabilitation . she was discharged on a regimen of warfarin and nifedipine , Follow-up cardiac catheterization on November 15, 1990, confirmed only mildly elevated pulmonary artery pressures and resistances (Table 1). Marked improvement in perfusion of both lower lobes was confirmed by repeat perfusion lung scan and pulmonary angiogram confirmed bilateral patenc y of lower lobe pulmonary arteries (Fig 3). When last seen nine months following her P-TEA, she exhibited minimal intention-tremor of her upper extremities . DISCUSSION
The treatment of isolated, chronic, thromboembolic P-HTN by P-TEA has now been well established ..-sHowever, few data are available as to the natural history of recurrent small tumor emboli from right atrial myxoma vis-a-vis the development of substantial P-HTN . much less treatment options when these two problems coexist. 1•• ,7 Our decision to perform initial myxoma excision without concomitant P-TEA was based on hemodynamic data which suggested a reactive pulmonary vascular bed and which did not suggest decompensated right heart failure . We felt that postoperative treatment with appropriately high-dose calcium-channel blocker therapy as suggested-w previously would allow a less risky procedure to be performed and that hemodynamic evaluation of the need for P-TEA could later be made with more certainty. Her subsequent episode of acute cor pulmonale emphasizes the danger in our initial approach. Therefore, it is suggested that a combined surgical approach as initial mangement is mandatory in the setting of severe secondary P-HTN in association with right atrial myxoma and can be expected to be associated with acceptably low mortality, as reported by others. II REFERENCES
Heath D, Mackinnon J. Pulmonary hypertension due to myxoma of the right atrium. Am Heart J 1964; 68 :227-35 2 Ch itwood WR, Lyerly HK, Sabiston DC. Surgical management of chronic pulmonary embolism . Ann Surg 1985; 201 :11-26 3 Moser KM, Auger WR, Fedullo PF. Chronic major-vessel thromboembolic pulmonary hypertension. Circulation 1990; 81:1735-43
F"" 'IIF :l, Post"p"rativ,' pulmonary cineangiogrum demonstrattng ....mpk-te rr-storation of flow t.. IM,th lower lohe pulmonary arteries.
302
4 Daily PO. Surgical management of chronic pulmonary embolism. In: Bergan J], Yao JST, eds. Venousdisorders. Philadelphia: WB Saunders Co, 1991:531-41 5 Daily PO, Demhitsky wa Peterson KL, Moser KM. Modifications of techniques and early results of pulmonary thromboendarterectomy for chronic pulmonary embolism . J Thorne CarPulmonary Hypertension and Right Atrial Myxoma (Heck. Gross. Houghton)
I
diovasc Surg 1987; 93:221-33
6 Keller H, Stegaru B, Buss 7 8
9
10
11
J,
Genth K, Heene D. Pulmonary tumor embolism and right atrial myxoma detected by twodimensional echocardiography. Am Heart J 1985; 110:881-84 Abdur RT, Gross H, Siegelman SS. Right atrial myxoma and pulmonary hypertension. NY State J Med 1970; 70:2996-3000 Riedel M, Stanek ~ Widimsky J, Prerovsky I. Longterm followup of patients with pulmonary thromboembolism: late prognosis and evolution of hemodynamic and respiratory data. Chest 1982; 81:151-58 Rich S, Brundage BH, Levy PS. The effect of vasodilator therapy on the clinical outcome of patients with primary pulmonary hypertension. Circulation 1985; 71:1191-96 Rich S, Brundage BH. High-dose calcium channel-blocking therapy for primary pulmonary hypertension: evidence for long term reduction in pulmonary arterial pressure and regression of right ventricular hypertrophy. Circulation 1987; 76:135-41 Daily PO, Dembitsky W~ Iversen S, Moser KM, Auger W Current early results of pulmonary thromboendarterectomy for chronic pulmonary embolism. Eur J Cardiothoracic Surg 1990; 4:117-23
J
In this report, we present a case where a patient needed 75 mg of captopril every 3 h for the control of his blood pressure. CASE REpORT
A 68-yr-old white man had a history of hypertension for over 15 years, and his BP had ranged from 200 to 218 mm Hg systolic and from 100 to 130 mm Hg diastolic. His peripheral venous plasma renin activity (PRA) was 10.4 nwmllh, and a renal scan showed a symmetric uptake and washout pro6le, with no asymmetry in renal size. Initially, the patient required 75 mg of captopril three times daily before meals and 50 mg of hydrochlorothiazide daily to lower his BP to 160180 mm Hg. His caring wife, who was trained to record B~ measured it for several days before and 2 to 3 h after each dose of captopril following any change in dosing schedule. After 6 rno, the BP rose again and fluctuated between 200190and 2101100 mm Hg. Review of the BP chart maintained by the wife indicated a rise only before the next dose was due. No cause for secondary resistance to captopril, such as a change in diet, salt intake, or physical activity, could be detected. There had been no problem with compliance, as indicated by pill counts. Instead of changing to another class of antihypertensive agent, as is customarily done in therapeutic failure, we elected to administer doses of captopril every 6 h, and the BP stabilized in the range of 156'70 to 170180 mm Hg. We had been encouraged to do so because of the lack of side effects from captopril in this patient. The BP went out of control during the next year, when a renal scan revealed markedly decreased size of and flow in the left kidney, and a renal arteriogram confirmed left renal artery occlusion. The left renal vein PRA was 130 nglml/h (right renal vein PRA, 70 nw ml/h, and inferior vena cava PRA, 71 ng/ml/h), As revascularization was not indicated for the severely shrunken left kidney, the patient was offered left nephrectomy, which he refused. Therapy with nifedipine was then added, and the BP remained under control for about 6 mo: however, the BP went out of control once again before each dose of captopril every 6 h, which was then increased to every 4 h, te, to 450 mglday (the maximum recommended dosage per the package insert). This lowered the BP only partially, into the range of 180180to 200190mm Hg. The dosing interval was then decreased to every 3 h, with careful monitoring of subjective and objective
Suman \Urdan, M.D.; Sa1ctipada Mookherjee, M.D.; and
Harold Smulyan, M.D.
The renovascular hypertension of an elderly patient, which was controlled initially with standard-dose therapy with captopril, later responded only to 75 mg given every 3 h without side effects. Changeover to another class of antihypertensives, as is recommmended in recalcitrant cases, was not needed. Convenient maintenance therapy with lisinopril (10 mg twice daily) has kept blood pressure under control for a year. (Chelt 1992; 102:303-04) *From the Sections of Cardiology (Drs. Vardan, Mookberjee, and Smulyan) and General Medicine (Dr. Vardan), Department of Medicine, Veterans Administration Medical and Health Science Centers, State University of New York, Syracuse. Reprint requests:Dr. \flrdan, VA MedicalCenter, 800 Irving Avenue,
Syracuse 13210-2799
200
CD
I
the treatment of hypertension, the usual recommended I"ndose of captopril is 25 to 50 mg taken at 8-h intervals.
Captoprll Administered Every Three Hours in Difficult-to-Control Hypertension
3 Hourly
PM = plasma renin activity
3 Hotxly
6 Hourly
4 Hourly
0-0 Systolic . - - . Diastole
J:
~~
~8 WJ: a: O
150~
SF a: ea:.
100
~~ WW
.. 0
o~
9 m
50
O~"" " " ' ' ' ' ' ' ' 2' ' ' . . -5I' ' ' ' .8. -I' ' 112 ' ' ' ' ' ' ' '_' -5' ' ' ' ' 8' -' ' ' "112 " ", ,' ' ' ' ' ' ' 5' ' ' ' ' 811 '_ 2 5
9 1 5 9 1 5 9 1 5 9 1
-PM- -NI- -PM- -AM-
~
-AM-
- PM-
TIME
10 4
-AM-
10 4
-PM-
10 4
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10
-PM-
7 101471015 ~-PM-
§
-AM-
FIGURE 1. Frequency of captopril administration and BP response in hypertensive patient. CHEST I 102 I 1 I JUL~
1992
303