Therapeutic balloon occlusion and pharmacologic therapy of a right-to-left atrial shunt produced by right ventricular infarction

November

1044

Brief

Communications

American

Heart

1989 Journal

I. Angiographic and hemodynamic data before and immediately after balloon angioplasty, and at follow-up

Table

Peak systolic at constriction Patient 1 2

3

Angiographic appearance

Hourglass Membrane Hourglass

Before

After

165

28

110

1.5

65

gradknl (mm Hgi --

After

30 18

11

18

18.5

IX. “0

10 13

15

15

1:). 15

62

In: Adams in infants,

FH, chil-

I;‘O//OUl-Up

Bullwm sm fmml

Before

REFERENCES

Benson LN. Aortic stenosis. GC, eds. Moss’ Heart disease

(mm) of he,qment

t’ollow-up

supravalvular AS. After balloon dilatation, peak systolic pressuregradient acrossthe stenosismarkedly decreased without significant changein cardiac index in two patients (Fig. 1). In these patients the diameter of the constricted segmentalso markedly improved. This included the patient with “elfin” facies who had hourglasstype of supravalvular AS (Fig. 2). In one patient the stenosiscould not be dilated despite attempts to dilate using a balloon with the same diameter as the normal ascendingaorta. Mild aortic regurgitation waspresentin onepatient. This did not increaseafter balloon dilatation. Reevaluation by M-mode, two-dimensional, and continuous wave Doppler echocardiography 6 weekslater in the two successfullydilated patients showedsustainedrelief of stenosis(peak aortic systolic flow velocity decreasedfrom 6.61 m/set to 2.96 m/set in the first and from 5.48m/set to 2.59 m/set in the second patient). Follow-up hemodynamic and angiographic reevaluation after 6 months in successfullydilated patients showedpersistent improvement, with no evidence of restenosis(Table I, Fig. 1). Both patients have shownsustained symptomatic improvement on long-term follow-up (18 months in patient No. 1 and 8 months in patient No. 2). Although surgery of supravalvular AS by patch aortoplasty is well established, this approach has associated mortality, morbidity, and sometimesmay not relieve the outflow tract obstruction completely.7 Balloon dilatation techniquesare increasinglybeing usedto relieve stenosisof valves and aorta. Experience with balloon dilatation of coarctation of the aorta and the aorta in arteritis indicates that fibromuscular lesions are amenable to balloon dilatation.8 Histologically localized supravalvular AS consists of intimal thickening, focal disorganization and degeneration of elastic fibers, together with fibrosis and increaseof smoothmusclein the media.gOur results indicate that balloon angioplasty can relieve supravalvular AS. The procedure is safe and the patient can be discharged early. Follow-up of successfully dilated patients shows sustainedrelief of stenosis.Inability to relieve obstruction by balloon dilatation in one adult patient, however, indicatesthat supravalvular AS can be a very rigid lesion.This could be due to extreme thickening of the aortic media. Although our experience is limited, it can be concluded that the balloon dilatation technique can be safely used asthe initial mode of treatment with goodresults in supravalvular AS.

1. Friedman WF, Emmanouilides

Iliametcjr (wnstrictecl

13

15 9 “0 -

andadolescents. Baltimore:The Williams& WilkinsCo, 1953:183.

dren

2. Choy M, Beekman RH, Rocchini AP, Crowley DC, Snider AR, Dick M, RosenthalA. Percutaneous balloonvalvuloplastyfor valvar aortic stenosis in infants and children. Am J Cardiol 1987;59:1010. < Perry SB, Keane JF, Lock JE. Interventional 7 catheterization in pediatric congenital and acquired heart disease. Am J Cardiol 1988;61:109G. 4. Suarezde Lezo J, Pan M, Sancho M, Herrera N, Arizon .J, Franc0 M, Concha M, Valles F, Romanos A. Percutaneous transluminal balloon dilatation for discrete subaortic steno sis. Am J Cardiol 1986;58:619. I5 Lababidi Z, Weinhaus L, Stoeckle H Jr, Walls JT. Transluminal balloon dilatation for discrete subaortic st,enosis. Am .J Cardiol 1987;57:423. 6. D’Orsogna L. Sandor GGS, Culham JAG, Patterson M. Successful balloon angioplasty of peripheral pulmonary stenosis in Williams syndrome. AM HEART J 1987;114:647. Flaker G, Teske D, Kilman J. Hosier D, Wooley C. Supravalvular aortic stenosis: a 20.year clinical perspective and experience with patch aortoplasty. Am J Cardiol 1983;51:256. Khalilullah M, Tyagi S, Lochan R. Yadav BS, Nair M, Ganbhir DS, Khanna SK. Percutaneous transluminal balloon angioplasty of the aorta in patients with aortit,is. Circulation 1987;76:597. Perou MC. Congenital supravalvular aortic stenosis. Arch Path01 1961:71:453.

Therapeutic balloon occlusion and pharmacologic therapy of a right-to-left atrial shunt produced by right ventricular infarction Thomas M. Broderick, MD, and JamesC. Dillon, MD. Indianapolis,

Ind.

The present patient is a 6%year-old white woman who developed new symptomsof shortnessof breath shortly after dischargefrom an outlying hospital. During this hospitalization, her treatment had beenfor unrelated problemsand From the Krannert Institute of Cardiology. ana University School of Medicine.

Department

of Medicine,

Indi-

Supported in part by the Herman C. Krannert Fund; by grants HL-06308 and HL-07182 from the National Heart, Lung, and Blood Institute of the National Institutes of Health, Bethesda, Md.: and by the American Heart Association, Inc.. Indiana Affiliate. Reprint requests: 926 U’. Michigan

414/15190

Thomas M. Broderick, MD, St., Indianapolis, IN 4622:X

University

Hospital

N-662.

Volume

118

Number

5. Part

Brief Communications

1

1. Anteroposterior projection. Contrast injection in the superior vena cava and right atrium demonstratesintratrial shunting and faint opacification of the left atrium. RA, Right atrium; ASD, atria1 septal defect. Fig.

Table

Fig. 2. Thirty-degree left anterior oblique projection of the right coronary artery showingcomplete obstruction after the first right ventricular branch. RCA, Right coronary artery; RV, right ventricular.

I. Arterial blood gasanalysis FlOp

Tine

Baseline(asymptomatic) Readmission (right atrial)

(day

forced

inspiratory

oxygen;

PC@, carbon

1)

21%

(day 9) (day 13)

Catheterization ASD occlusion ASD occlusion ASD occlusion ASD open (1 hour post) p-blocker (30 min post) Discharge (right atrial) FIO2,

1045

100% 13) 100% 15) 50% 16) 6 L 16) 507 16) 6 L (day 23) pressure;

Poq, arterial

included the addition of enalapril to her antihypertensive regimen. Her shortnessof breath progressed,prompting her readmission5 days later (day 9) with severehypoxemia, notably changedfrom her baselinearterial blood gasvalues(day 1) (Table I). Her chest x-ray film wasunrevealing and additional pulmonary evaluation included a low probability ventilation-perfusion lung scan and pulmonary function tests consistent with a mild combined restrictive and obstructive defect. Her electrocardiogramshowednew ST segmentelevation in the inferior leadsimposedon an age-undeterminedmyocardial infarction noted during the previous admission.No prior history of myocardial infarction could be elicited. An antecedent 6-month history of exertional angina waspresent. Despitethe absenceof chest pain, cardiac enzyme determinations confirmed a recent infarction with the peak creatine kinase (CK) (754~2% MB isoenzyme) noted on the secondadmission.Therapy included supplemental oxygen, diuretics, and discontinuation of her P-blocker. She wastransferred to our institution on the third hospital day with progressivehypoxemia.

PC@?

pf&?

7.47 7.49

24 24 26 34 34 32 21 33 37

147 41 45 71 112 64 49 93 79

7.35 7.41 7.43 7.43 7.57 7.42 7.49

(day (day (day (day (day

dioxide

PH

oxygen

p~ssure;

o;, sat, percent

oxygen

saturation;

%snt

ASD,

atria1

septal

defect,

An echocardiogramdemonstratedan inferoposterior infarct, mild right atria1 and right ventricular dilatation, and mild right ventricular hypokinesis.Echocardiographic contrast injection revealed a large right-to-left shunt at the atria1 level. The atria1 septum could not be well visualized and so a defect wasnot seen.Cardiac catheterization was performed the day of transfer. Tables I and II contain the hemodynamic and oximetric data. Ventriculography confirmed the inferoposterior wall motion abnormality. A contrast injection in the right atrium showedright-to-left shunting through an atria1 septaldefect (ASD) (Fig. 1) and a mildly hypokinetic right ventricle. Coronary anatomy wasnormal except for total occlusionof the right coronary artery distal to the first right ventricular branch (Fig. 2). Dye curves were performed to exclude any significant leftto-right shunt and they establisheda right-to-left shunt at the atria1 level. Therapeutic occlusionof the ASD wasperformed at the time of the initial catheterization with a contrast-filled pulmonary artery catheter withdrawn until it was snugin

November

Brief Communications

1046

American

Table

II. Hemodynamic Day

LV

AA 132194-108 CO 3.2 L/min HR 116/min RA, Right atrial; RV, right ventricular: monary capillary wedge pressure; LA, aortic arch; CO, cardiac output: HR, *Day 13: initial catheterization data. tDay 16: repeat hemodynamic data pulmonary artery catheter and atria1

Anteroposterior projection. Diagnostic pulmonary artery catheter placed normally in the right pulmonary artery. In the center, contrast-filled pulmonary artery catheter in the left atrium and pulled snug into the atria1 septal defect.

the defect (Fig. 3). The size of the defect was estimated to be 8 to 10 mm, based upon the normal inflated diameter of the balloon. ASD occlusion resulted in a significant improvement in the oxygenation, but not in complete elimination of all shunting. The balloon catheter was left in place for 3 days, while the angiotensin-converting enzyme (ACE) inhibitor and diuretic were discontinued and the patient was actively rehydrated. Prior to the ASD balloon removal, the hemodynamics were more consistent with a typical right ventricular infarction pattern despite the improvement in the patient’s oxygenation (Table II). ASD balloon removal was accompanied by worsening of the right-to-left shunting, as evidenced by deteriorating oxygenation. Pharmacologic therapy was initiated with ultrashort-acting intravenous P-blocker therapy, producing improvement in oxygenation over the next 30 minutes. No follow-up hemodynamic data are available following the intravenous p-blocker therapy. Subsequent long-term pblocker therapy produced continued clinical improvement and the patient was discharged the following week with a POZof 74 (95 % saturation) on room air. She is subsequently doing well at 3 months, with a POTof 95 on room air. The patient apparently had a patent foramen ovale or ASD without previous clinical manifestation. The significant right-to-left shunt is temporally related to several events. The most important hemodynamic changes were produced by right ventricular infarction and/or ischemia. Afterload reduction with an ACE inhibitor and preload reduction with a diuretic may have further unfavorably affected the hem0dynamics.i The increase in right-sided heart pressures normally seen with right ventricular in-

Day

1st

27/10

16

13*

RV 2014 PA 20/10-15 PCWP LA3

3.

1989 Journal

data

Ra 5

Fig.

Heart

8

3 11814 150/90 110 6.3 L/mm 115/min PA, pulmonary artery; PCWP, pulleft atrium; LV. left ventricular; AA. heart rate. immediately prior to removal of the septal defect-occluding catheter.

farction or ischemia has been demonstrated to be related to decreased right ventricular compliance and increasing diastolic volumes.“-4 A secondary effect of the diminished stroke volume of the right ventricle can also include lower preload on the left ventricle.3l 4 Interestingly, the patient did not demonstrate the significant rise in the right ventricular and right atria1 pressures or the characteristic changes in waveforms that are often seen with a right ventricular infarction4 The absence of these changes is probably related to relative volume depletion, tachycardia, lack of significant chamber dilation, and some equalization of pressures through intra-atria1 shunting. The patient was able to develop shunting across the atria1 septum because of a small atria1 pressure difference and presumed increased resistance to filling in the right side of the heart. The relative contribution of decreased ventricular compliance is further confirmed by the hemodynamics obtained after active rehydration. At that point, shunting across the septum decreased despite an apparent rise in the atria1 pressure difference. Presumably, the wall stiffness associated with ischemia and/or infarction diminished during this time period despite the volume administration and resultant rise in pressure difference. Therapeutic balloon occlusion was felt to provide a temporary means to diminish shunting while rehydration, removal of vasodilator therapy, and resolution of the ventricular stiffness related to the acute right ventricular infarction were instituted. The benefit of these other interventions was supported by the gradual but mild improvement in oxygenation during the period of balloon occlusion. The effectiveness of the balloon catheter occlusion of the ASD was established by the prompt improvement of oxygenation at placement, and was confirmed by the deterioration following removal. P-blocker therapy was chosen after balloon removal because of the bedside observation that the patient’s oxygen saturation improved during periods of sleep when the blood pressure and heart rate were relatively reduced. Although the affects of p-blockers on ventricular function and com-

Volume

118

Number

5, Part

pliance have been described for the left ventricle,5l6 it is likely that many of these benefits apply to the right ventricle as well. Lowering the heart rate has previously been demonstrated to improve the filling characteristics of the ventricular chambers.5 The reduction of the heart rate could also decrease oxygen consumption and alleviate any additional noncompliance that might result from right ventricular ischemia in the periinfarct regions.2 Although the negative inotropic effects of the P-blockers could have detrimental effects on right ventricular performance, the Starling forces provided by the increased filling at slower heart rates could foreshadow any negative inotropic affects of the drug.7 Additional benefits of @-blockers on ventricular compliance have been demonstrated apart from the changes in ventricular filling or contractility.5 The effectiveness of this therapy was remarkable and manifested in only a short period of time by an improvement in oxygenation coincident with a reduction in heart rate and blood pressure. Simultaneous hemodynamic data are unavailable to confirm the postulated mechanism of action. Usually, improvement in the right ventricular compliance after an acute infarct is gradual over days to weeks.8 The more rapid improvement seen with P-blockers could be supportive of ischemic noncompliance, but incomplete resolution implies a less reversible component that can probably be attributed to right ventricular infarction. Right-to-left shunting has been previously demonstrated across the atria1 septum in patients with acute right ventricular infarcts.g In addition, a recently published case report describes the use of a Rashkind septostomy balloon to temporarily occlude a patent foramen ovale in attempts to improve oxygenation, lo This case differs in several respects from the previously published case report, since the patient described here had less right ventricular systolic impairment and greater effectiveness of the occluding balloon catheter. No previous description of successful pharmacologic correction of the hemodynamic derangements of right ventricular infarction with @-blockers has been published. REFERENCES

1.

2.

3. 4.

5.

6. 7.

Brief Communications

1

Gaasch WH, Levine HJ, Quinones MA, Alexander JK. Left ventricular compliance: mechanisms and clinical implications. Am J Cardiol 1976;38:645-53. Coma-Covella I, Lopez-Sendon J. Ventricular compliance in ischemic right ventricular dysfunction. Am J Cardiol 1980; 45:555-61. Cohn JN, Guiha NH, Broden MI, Lindus CJ. Right ventricular infarction. Am J Cardiol 1974;33:209-14. Lloyd EA, Gersh BJ, Kennely BM. Hemodynamic spectrum of “dominant” right ventricular infarction in 19 patients. Am J Cardiol 1981;48:1016-21. Coltant DJ, Alderman EL, Robinson SC, Harrison DC. Effect of propanolol on left ventricular function, segmental wall motion, and diastolic pressure-volume relation in man. Br Heart J 1975;35:357-61. Lewes BS, Gotsman MS. Current concepts of left ventricular relaxation and compliance. AM HEART j 1980;99:101-12. Glower DD. Snratt JA. Snow ND. et al. Linearitv of the Frank-Starling’relationship in the intact heart: the concept of preload maintainable stroke work. Circulation 1985;71:9941099.

8. Lew

AS, Isner

JM.

Right

ventricular

infarction.

1047

Cardiovasc

Clin 1982;13:203-17. 9. Manno BV, Bemis CE, Carver J, Mintz GS. Right ventricular infarction complicated by right-to-left shunt. J Am Co11 Cardiol 1983;1:554-7. 10. Upstrom EL, Kern MJ, Mezei L, Mrosek D, Labovitz A. Balloon catheter closure of patent foramen ovale complicating right ventricular infarction: improvement of hypoxia and intracardiac venous shunting. AM HEAW J 1988;116:1092-7.

Right atrial papillary fibroelastoma: Diagnosis by transthoracic and transesophageal echocardiography percutaneous transvenous biopsy

and

Matthew E. Schwinger, MD,a Edward Katz, MD,” Heidrun Rotterdam, MD,b JamesSlater, MD, Edwin C. Weiss, MD,a and Itzhak Kronzon, MD.a New York, N.Y.

Papillary fibroelastomais an uncommoncardiac tumor. It is rarely diagnosedduring life. We report the antemortem detection and characterization of a right atria1 papillary fibroelastomaby transthoracic and transesophagealechocardiography. Tissue was obtained by percutaneous transvenous biopsy to establishthe diagnosis. An 83-year-old woman wasadmitted to our hospital for evaluation of progressivedyspneaand fatigue of 3 months’ duration. She had been hospitalized elsewhere1 month earlier for acute shortnessof breath and wasfound to be in rapid atria1fibrillation. Her chestx-ray film did not reveal any infiltrates or vascular congestion.Analysis of arterial blood gasesrevealed marked hypoxia (POT = 5ii mm Hg). Her respiratory symptoms were attributed to airway diseasesecondary to her 75 pack-year smoking history. She was treated with albuterol, aminophylline, and digoxin, with improvement in her symptoms and POT. After discharge,her respiratory symptomsprogressively worsened. There wasno chestpain, orthopnea, paroxysmal nocturnal dyspnea, or pedal edema. Her past medical history was significant for hypertension and a right below-kneeamputation in 1980secondaryto an arterial embolusof uncertain etiology. On admissionto our hospital, physical examination

revealed

a well-appearing

female

in no acute

distress.

The pulse was 120 beats/min and irregular. The blood pressurewas170/110mm Hg. Auscultation of the chest revealed scateredralesat the bases.Auscultation of the heart wasnormal. There wasno jugular venousdistention or peripheral edema. The electrocardiogram revealed atria1

From the Departments Medical Center. Reprint requests: NY 10016.

414115189

Itzhak

of Medicin? Kronzon,

and Pathology,b MD,

560 First

New Ave.-Suite

York

University

:!E, New York.