* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Download Use of Contrast Echocardiography for Evaluation of
Survey
Document related concepts
Heart failure wikipedia , lookup
Cardiac contractility modulation wikipedia , lookup
Cardiac surgery wikipedia , lookup
Management of acute coronary syndrome wikipedia , lookup
Echocardiography wikipedia , lookup
Hypertrophic cardiomyopathy wikipedia , lookup
Lutembacher's syndrome wikipedia , lookup
Mitral insufficiency wikipedia , lookup
Ventricular fibrillation wikipedia , lookup
Atrial septal defect wikipedia , lookup
Dextro-Transposition of the great arteries wikipedia , lookup
Quantium Medical Cardiac Output wikipedia , lookup
Arrhythmogenic right ventricular dysplasia wikipedia , lookup
Transcript
Use of Contrast Echocardiography for Evaluation of Right Ventricular Hemodynamics in the Presence of Ventricular Septal Defects GERALD A. SERWER, M.D., BRENDA E. ARMSTRONG, M.D., PAGE A.W. ANDERSON, M.D., DOUGLAS SHERMAN, B.A., D. WOODROw BENSON, JR., M.D., PH.D., SAM B. EDWARDS, M.D. Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 SUMMARY Intracardiac blood flow is altered in the presence of a ventricular septal defect (VSD), with different sizes of defects producing different flow patterns that can be visualized by peripheral injection contrast echocardiography. The utility of these patterns in allowing estimation of right ventricular pressure and resistance to ejection of blood from the right ventricle (RV) was investigated. Forty-four patients underwent 46 contrast echocardiographic procedures, all within 24 hours before cardiac catheterization. All patients were placed in one of four groups based on catheterization findings. Group I, which consisted of patients with no VSD, or a small VSD with a pulmonary-to-systemic flow ratio (Qp/Qs) < 1.5:1 and right ventricular pressure < one third systemic, had echo-dense material appear in the RV only. Group II consisted of those with moderate-sized VSDs with Qp/Qs 2 2.5:1 and right ventricular pressure 60-80% systemic; in these patients echodense material appeared in the RV and in the left ventricle (LV) anterior to the mitral valve. The contrast appeared in the LV in early diastole and cleared from the LV in systole, with none appearing in the aorta or left atrium. Time from the preceding QRS to appearance of echo-dense material in the LV divided by the R-R interval (appearance time to R-R interval ratio) was 0.62 0.69. Group III consisted of those with large VSDs, Qp/Qs > 3:1 and systemic right ventricular pressure with low pulmonary vascular resistance and no pulmonary stenosis. These patients had appearance of echo-dense material in the LV during early diastole anterior to the mitral valve as in group II, but the material remained in the LV to be ejected into the aorta. Appearance time to R-R interval ratio was the same as in group II. Group IV consisted of patients with tetrology of Fallot and one patient with a large VSD and increased pulmonary vascular resistance. Echo-dense material again appeared in the LV early in diastole and was ejected into the aorta during systole. The appearance time/R-R ratio was significantly less than in groups II or III, allowing a clear separation between those patients in group III from group IV. Contrast echocardiography allows estimation of the right ventricular pressure and evaluation of increased resistance to pulmonary flow due to either pulmonary stenosis (PS) or increased pulmonary vascular resistance in the presence of VSDs. This technique also provides a minimally invasive method which may be useful for serial evaluation of such patients, and will permit early detection of developing PS, and possibly increasing pulmonary vascular resistance. Decreasing right ventricular pressure due to spontaneous closure of the VSD may also be detected by this technique. - CONTRAST ECHOCARDIOGRAPHY, initially introduced clinically by Gramiak et al.,' has been used previously to identify intracardiac structures validating echocardiographic patterns,2' 3 to identify various congenital anomalies4-7 and to demonstrate valvular insufficiency.4 I Furthermore, as the echodense material is easily traced through the cardiac chambers, significant information concerning intracardiac hemodynamic flow patterns can be obtained. In this manner, right-to-left shunting8 and leftto-right shunting9 can be demonstrated. However, the emphasis in these previous studies was on the identification of structural defects rather than on the underlying hemodynamic states that existed in the various forms of congenital heart disease evaluated. In this study, children with various sizes of ventricular septal defects (VSDs), including large VSDs with associated pulmonary stenosis or elevated pulmonary vascular resistance, were evaluated using the technique of peripheral injection contrast echocardiography. In each patient the flow patterns were cor- related with the hemodynamic status observed at cardiac catheterization. This correlation demonstrated that using specific contrast echocardiographic patterns, the intracardiac pressure-flow relationships could be determined accurately. In this manner, conclusions concerning the right ventricular and pulmonary vascular hemodynamic states can be drawn and serially re-evaluated in patients with VSDs. Methods Patient Population Forty-four patients between the ages of 2 weeks and 16 years comprised the study group. Two patients each underwent two echocardiographic and catheterization studies approximately one year apart. Based on hemodynamic data obtained at catheterization, all patients were grouped into one of four categories. No patient included in this study had an atrial septal defect or a communication at the great vessel level. Catheterization data is presented in table 1. I From the Division of Pediatric Cardiology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina. Supported in part by NIH grants HLl 1307, HL20677 and HLO7lOl. Address for reprints: Gerard A. Serwer, M.D., P.O. Box 3090, Duke University Medical Center, Durham, North Carolina 27710. Received November 7, 1977; revision accepted March 31, 1978. Group I Patients included in this group (mean age 3.8 years ± 1.1 SEM) had either no VSD (n = 9) or a small VSD with pulmonary to systemic flow (Qp/Qs) less than 1.5:1 (n = 8). All but one (patient DR) with small VSDs had peak right ventricular pressures less 327 VOL 58, No 2, AUGUST 1978 CIRCULATION 328 TABLE 1. Catheterization and Echocardiographic Patient Data Age Pt Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 Group I 6 wks CG DR 6 wks 9 mos SG 4 yrs RD 1 yr JK KT 6 yrs TS 16 yrs 2 yrs DH 3 mos JM 7 mos TH 2 yrs BM 3 yrs CF 3 yrs SB 3 yrs TK 4 yrs MH 5 yrs LS 14 yrs KP RV press LV press Qp Qs Ao 02 sat 0.31 0.42 0.26 0.33 1.4 1.4 1.3 1.3 1.1 1.1 1.4 97 94 96 94 98 95 97 1.1 1.0 1.0 1.0 1.0 1.0 1.0 1.0 1.0 1.0 95 93 94 95 0.28 0.28 0.25 0.33 0.40 0.35 0.34 0.63 0.73 0.50 0.27 0.40 0.23 Rp R, (Units/m2) Rs 1.7 2.4 1.1 2.2 1.9 1.1 1.2 3.3 0.11 0.15 0.15 0.10 98 95 98 1.9 1.4 1.1 1.8 1.8 2.8 1.3 2.3 2.6 94 95 94 95 1.6 3.4 1.3 1.0 95 1.3 1.4 1.5 4.9 1.5 95 95 95 Appearance tpie R-R (sec- Interval 0.25 0.25 0.28 0.27 0.25 0.22 0.30 0.25 0.28 0.38 0.37 0.45 0.43 0.36 0.35 0.45 0.40 0.47 0.67 0.63 0.60 0.26 0.25 0.26 0.27 0.29 0.34 0.27 0.39 0.36 0.38 0.39 0.47 0.54 0.43 0.67 0.69 0.68 0.69 0.62 0.63 0.63 AT R-R 0.17 0.17 0.10 0.15 Group II CC HE ES CH MD RG DH GS MC* 2 2 4 4 8 8 4 9 11 wks wks wks wks wks wks mos mos mos 0.80 0.65 0.70 0.65 0.65 0.63 0.60 0.76 0.64 5.6 2.5 4.6 7.7 2.9 4.0 4.5 3.0 95 2.5 94 5.0 3.2 93 92 3.4 4.0 6.5 3.2 3.0 94 93 93 0.07 0.18 0.09 0.07 0.07 0.14 0.12 0.32 0.17 0.66 0.68 0.62 0.63 0.69 0.63 Group III MC RG JP JT SL Jp* TA 2 4 6 2 2 11 2 wks wks wks mos mos mos yrs 1.0 1.0 1.0 1.0 1.0 1.0 1.0 1.4 1.5 4.9 2.0 3.6 5.0 2.3 88 87 93 92 0.06 0.12 0.28 0.18 0.10 0.24 0.15 Group IV 0.23 3 mos 1.0 84 0.58 0.24 79 0.71 1.0 5 mos 0.23 92 0.87 6 mos 1.0 0.29 58 0.26 17 mos 1.0 0.26 2 yrs 69 0.54 1.0 0.32 77 0.62 3 yrs 1.0 0.27 3 yrs 56 0.33 1.0 0.31 3 yrs 77 1.0 0.59 0.29 78 3 yrs 0.43 1.0 0.31 82 0.70 6 yrs 1.0 0.31 85 0.60 1.0 9 yrs 0.40 84 15 yrs 0.41 1.0 0.38 0.95 16.7 13 yrs 83 1.0 1.0 *Indicates data of the two follow-up studies. tlndicates the patient with a VSD and increased pulmonary vascular resistance Abbreviations: Pt = patient; RV press/LV press = ratio of the RV to LV peak systolic pressure; Qp/Qs to systemic blood flow; Ao 02 sat = aortic oxygen saturation; Rp = pulmonary vascular resistance; RP/Rs VJ MS AB BM MF ML FA CS JY DJ VW CK GVt vascular resistance to the systemic vascular resistance; AT/R-R to the eiectrocardiographic R-R interval ratio. than one-third that of the systemic pressure. Those patients with no VSD had valvular pulmonary stenosis. This ranged from mild to moderate in severity. None of the patients in this group had any evidence of rightto-left shunting by catheterization techniques. = the echo-dense material appearance 0.39 0.41 0.41 0.49 0.44 0.54 0.48 0.54 0.49 0.58 0.56 0.69 0.81 t.me ratio of the 0.59 0.59 0.56 0.59 0.59 0.59 0.56 0.57 0.59 0.53 0.55 0.58 0.47 pulmonary ratio of the pulmonary as defined in the text Group II These patients (n = 9, mean age 0.32 ± 0.11 years) had moderate sized membranous VSDs with a Qp/Qs ratio . 2.5: 1. All patients had right ventricular pressures 60%-80% of the systemic pressure. None CONTRAST ECHO IN VSD/Serwer et al. had pulmonary stenosis, and all had a pulmonary vascular resistance less than 5 units/M2 (1 unit = 1 mm Hg/1/min). None had evidence of right-to-left shunting as demonstrated by either Cardio-Green indicator dilution dye curve, Fick data or angiographic techniques. However, right ventricular cineangiocardiography did show diastolic flow of radiographic contrast material into the left ventricle. With ventricular systole, contrast material returned to the right ventricle, and none was ever visualized in the aorta. Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 Group III These patients (n = 7, mean age 0.54 ± 0.29 years) had large VSDs, with Qp/Qs ratios > 3: 1 and systemic pressure in the right ventricle. No patient had pulmonary stenosis. All had a pulmonary vascular resistance < 5 units/M2. Cineangiocardiography demonstrated a large left-to-right shunt at the ventricular level. In addition, by right ventricular cineangiography, a small right-to-left ventricular shunt was noted with radiographic contrast material appearing in the aorta. Two of the six patients had aortic desaturation indicative of a small right-to-left shunt (< 25%). The two older patients had CardioGreen dye curves performed with injection into the right ventricle showing small (< 20%) right-to-left shunts. Group IV Of the patients in this group (n = 13, mean age 4.6 ± 1.3 years), 12 had tetralogy of Fallot with rightto-left ventricular level shunting. This was substantiated by right ventricular cineangiocardiography and aortic desaturation. The remaining patient had an isolated VSD with an elevated pulmonary vascular resistance of 16.7 units/m2. The right ventricular and pulmonary artery peak pressures were systemic. There was aortic desaturation and right-to-left shunting demonstrated by right ventricular cineangiography. Echocardiographic Examination Patients underwent peripheral injection contrast echocardiography utilizing the microcavitation effect'0 within 24 hours before cardiac catheterization. Injections of either isotonic saline or the patient's blood were made into either a leg or arm peripheral vein. The injections did not exceed 2 ml of injectate per injection. Injections which produced the most reproducible records were made through either an 18 or 20 gauge indwelling catheter. Smaller catheters or needles produced inconsistent echo-dense material. Larger catheters also did not produce satisfactory results due to inconsistent production of echo-dense material. Echocardiographic examinations were performed with the patient in the supine position with the EchoCardioVisor echocardiograph interfaced with a Honeywell LS-6 strip chart recorder. A 4.5 mHz nonfocused transducer was used for all studies. Records were recorded at paper speeds of 50 mm/sec and 100 mm/sec. Initial injections were recorded at a paper 329 speed of 50 mm/sec. If echo-dense material was then found to be present in the left ventricle, repeat injections were recorded at a paper speed of 100 mm/sec. for measurement of the time intervals described below. Timing marks were inscribed on the paper every 500 msec. Time mark calibration with an external time base generator was performed periodically to ensure accuracy of the internal time base generator. Two echocardiographic views were utilized for each patient. Position #1 visualized the right ventricular cavity, septum, left ventricular cavity and mitral valve. Position #2 visualized the right ventricular outflow tract, aorta and left atrium. The reject control was initially set at its minimal setting and the time gain compensation controls adjusted to produce an adequate echocardiographic record. The reject control was gradually increased to the point that the overload phenomenon, as described by Valdes-Cruz et al.,8 9 was eliminated while still detecting echo-dense material produced by the injection. The patients' electrocardiograms were recorded simultaneously for timing purposes. Diagnostic records were obtained on all patients examined. All records were independently reviewed by at least three of the authors, and the results were collated before cardiac catheterization. All records were assessed for 1) presence or absence of echo-dense material in each chamber, and 2) the time of appearance of echo-dense material in the left ventricle. This appearance time was measured from the onset of the preceding QRS to the initial appearance of the echo-dense material in the left ventricle measured at the level of the left ventricular septal endocardium (fig. 1). This value was expressed both in absolute time (seconds) and as a fraction of the R-R interval. This measurement was made on the first cardiac cycle following the appearance of echo-dense material in the right ventricle. The echo-dense material was allowed to clear from the heart before the injection was repeated. A minimum of three determinations were performed for each patient with the results averaged. It is emphasized that this measurement was made at the first appearance of echo-dense material in the left ventricle during the first cardiac cycle following its arrival in the right ventricle. Thus, there was no residual echo-dense material in the left ventricle preceding the examined beat to obscure the correct point for measurement. Statistical analyses were performed using the Student t test. Catheterization Techniques Hemodynamic data was obtained at routine cardiac catheterization performed under sedation with meperidine, promethazine and chlorpromazine. Pressure data was obtained with a fluid-filled catheter system before angiography. The zero reference was set at the mid-chest level. Left and right ventricular pressures were measured with the same cathetertransducer system in quick succession (< 5 beats) via either a pullback across the VSD or introduction of the venous catheter into the left heart via a probe pa- CIRCULATION 330 Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 ,~~~~~~~~~~~2 VOL 58, No 2, AUGUST 1978 ............_- FIGURE 1. Record from patient in Group III showing the method of measurement of the echo-dense material appearance time. A T appearance time; R-R = electrocardiographic R-R interval. Paper speed is 100 mm/sec. tent foramen ovale. Pulmonary and systemic blood flows were calculated utilizing Fick techniques. All blood samples were obtained in rapid succession before angiography and 02 consumption was assumed based upon the work of Fixler et al.14 Pulmonary and systemic resistance was calculated from the pressure and flow data expressed both in absolute terms (units/m2) and as a ratio of pulmonary to systemic resistance (Rp/Rs). Presence of right-to-left and leftto-right shunting was evaluated by utilizing Fick techniques, indicator dilution Cardio-Green dye curves, radionuclide techniques and cineangiocardiography. Shunting detected by cineangiocardiography was evaluated for only those beats with normal conduction. Thus, the effect of premature ventricular contractions was eliminated. All data were independently reviewed by at least two authors without knowledge of the echocardiographic results. Results Group I Contrast echocardiography in patients of this group (fig. 2) demonstrated the appearance of echo-dense material only within the right ventricular cavity and right ventricular outflow tract. No echo-dense material was seen within the left atrium, left ventricle or the aorta. All patients, whether they had an intact ventricular septum or a small VSD, exhibited the same contrast echocardiographic pattern. Group 11 Contrast echocardiography (fig. 3) demonstrated the initial appearance of the echo-dense material within the right ventricular cavity. During the first ventricular diastole following appearance of the echodense material in the right ventricle, this echo-dense material appeared in the left ventricular cavity anterior to the mitral valve at the peak of the mitral valve opening. This was a consistent finding in all patients of this group. With the onset of ventricular systole and closure of the mitral valve, this echo-dense material cleared from the left ventricular cavity. Moreover, no echo-dense material ever appeared in the aorta or left atrium. This correlates with the results of the right ventricular cineangiogram in which there was opacification of the left ventricle during diastole with no radiopaque material appeating in either the left atrium or aorta. The interval from the QRS to the left ventricular appearance of the echodense material (echo-dense appearance time) varied from 0.22-0.30 seconds, depending on the heart rate (table 1). The ratio of the appearance time to the R-R interval (appearance time/R-R interval ratio) varied from 0.62-0.69, mean 0.65 + 0.01 (sEM). There was no statistical correlation of this ratio with heart rate for the patients in this group, P > 0.5 (fig. 4). Group IlI Contrast echocardiography (fig. 5) demonstrated initial appearance in the right ventricle followed by CONTRAST ECHO IN VSD/Serwer et al. 331 A. ,, * -~ ~~~yq½ a &'4~~qr* ~ .<uRC"1_ *t\LV T ,''', Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 * *J&a Zr _ 7f r,; .ejw <.4w i8' oi ti r%*... w tAt... * -t f D. A0 -LA w.~~~~~~~~~~ _ FIGURE 2. Record from a patient in Group l. Panel A shows echo-dense material only in the R Vfirst appearing at the arrow. Panel B shows echo-dense material only within the right ventricular outflow tract. Simultaneous ECG is shown only for panel A. R V right ventricle; LV left ventricle; MV mitral valve; R VOFT = right ventricular outflow tract; Ao = aorta; LA = left atrium. = appearance of echo-dense material in the left ventricle during the first ventricular diastole following its appearance in the right ventricle. Echo-dense material appeared in the left ventricle at the peak of the mitral valve opening anterior to the mitral valve echo (fig. 5A). In contrast to group II, it remained in the left ventricle during ventricular systole and subsequently appeared in the aorta (fig. 5B). Echo-dense appearance time ranged from 0.25-0.34 seconds with the appearance time/R-R interval ratio ranging from 0.62 to 0.69, mean 0.66 ± 0.01. There was no significant difference in this ratio between groups IL and III (fig. 6). Again, for those patients in group III, there was no relationship to heart rate (P > 0.5, fig. 4). 332 Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 w r77, ..' VOL 58, No 2, AUGUST 1978 CIRCULATION '4 ey~~; @.'s~\' K;2h&j '4 1zu. t - ' . FIGURE 3. Recordfrom a patient in Group II with echo-dense material appearing in the R Vand L Vat the arrow. Note that there is no echo-dense material in the L V during systole. R V = right ventricle; L V - left ventricle; MV = mitral valve. oup IV Two patients, JP and MC, in this group each underwent a second study. JP continued to exhibit a flow pattern characteristic of group III patients. MC, at restudy, exhibited a flow pattern characteristic of group II patients. The catheterization data did show a decrease in the right ventricular pressure of MC and no change in the hemodynamic status of JP. .70 Pontrast echocardiography (fig. 7) demonstrated tial appearance of echo-dense material in the right itricle, which then appeared in the left ventricle Lerior to the mitral valve during the first ventricular Lstole in all patients; just as in the preceding two ups. It remained in the left ventricular cavity dur- O O K0 0 S S 0 6 0 09 0 c AA A "I S FIGURE 4. Plot of heart rate (beats/min) vs appearance time/R-R interval ratio. There is no correlation of these variables for patients within each group. The follow-up data of the two patients studied twice is not included. A Ad A .57 a A A A A Cl CL cr * Group ! o Group M a Group I A6 .45 I I 75 125 Heart Rate I 175 CONTRAST ECHO IN VSD/Serwer et al. 333 A. o ihv¾s RV~ It j %..-0_ _ <9~ss_s.... ¶-e Z% t t c. tqP 8- °_ ^*f,2t ,.. t. f Ut LV %~ t l ~t MV \, k Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 k k -jeo-...,e 0 B. --- a A RVOFT 'I '" 11 Ibml . #),.,w t A , v : . * -. .. 1*0.. I\''*, OSf *N k , A It 4 --l' f -~~~~~~~~*I Ad. of OM_ LA _. -t '_. A >o *t ;~~~~~~~~~~~~~~~~~~~~ FIGURE 5. Recordfrom apatientin Group IIIshowingappearance of echo-densematerialin theRV, LV, right ventricular outflow tract and aorta as indicated by the arrow. Panel A shows appearance ofecho-dense material in the L Vduring diastole and its remaining there during systole. Panel B shows appearance of echodense material initially in the right ventricular outflow tract and then in the aorta. The ECG has been omitted from panel B. R V = right ventricle; L V = left ventricle; MV = mitral valve; R VOFT - right ventricle outflow tract; Ao = aorta; LA - left atrium. ing ventricular systole and was visualized within the as in group III. Echo-dense material appearance time ranged from 0.23-0.40 seconds with the appearance time/R-R interval ratio ranging from 0.47-0.59, mean 0.57 ± 0.01. The ratio was statistically different (P < 0.001) from the values found for groups II and III patients (fig. 6). For patients in this group, the ratio was also independent of heart rate, as in groups II and III (fig. 4). The effect of heart rate on this ratio must be evaluated only aorta within each group. Combining groups II, III and IV indicates an apparent correlation of this ratio with heart rate. This is because the patients in groups II and III are significantly younger than those patients in group IV. Therefore, group II and III patients have higher heart rates than those patients in group IV, by virtue of age, and larger ratios by virtue of their different hemodynamic status. This leads to the false impression that the ratio increases with increasing heart rate. t VOL 58, No 2, AUGUST 1978 CIRCULATION 334 0.70 * S ~ ~~~~~~~ * es 0~~~~~~~~~ * i 0.65 0.65~~~~~ c *" 05 0) *:0 i 0.66 c : w E } 0.57 0 0) c 0) 0. S 0.45 I Group E - -1 Group m Group I7 Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 FIGURE 6. Plot of appearance time/R-R interval ratio of each group. The follow-up data of the two patients studied twice as not been included. The brackets indicate the standard error of the mean; the mean value is indicated by the number. There is no significant difference between Groups II and III. There is a highly significant difference between Groups II and III and Group IV (P< 0.001). Discussion In this study the patterns of intracardiac blood flow, demonstrated by peripheral injection contrast echocardiography, showed clear differences which were directly related to the underlying hemodynamic status. Specifically, the patterns demonstrated reflected alterations in the right ventricular hemodynamics as influenced by the presence and size of the VSD and the degree of obstruction to pulmonary blood flow secondary to either pulmonary stenosis or, in one instance, elevated pulmonary vascular resistance. The four groups represented were easily separated by this technique and represent significantly different hemodynamic situations. As a result, the hemodynamic significance of a VSD can be assessed and subsequent changes in a patient's hemodynamic state can be monitored using contrast echocardiography. In patients possessing either intact ventricular septa or small VSDs, (group I), there was no evidence by contrast echocardiography of intracardiac shunting from the right ventricle to the left ventricle. These results were confirmed by cardiac catheterization techniques that demonstrated no intracardiac blood flow from the right to the left ventricle. All patients who had VSDs in this group had low right ventricular pressures with small left-to-right shunts at catheterization. In group II patients, moderate sized VSDs with moderate elevation of the right ventricular pressure, there was a transient flow of blood from the right to the left ventricle that could readily and easily be identified by contrast echocardiography. The patients in this group were easily separated from those in group I. There was a flow of echo-dense material from the right to the left ventricle even though there was no pulmonic stenosis or elevation of the pulmonary vascular resistance present. This is a particularly important point, in that previous descriptions of contrast echocardiography have reported such a right ventricular to left ventricular flow only in the presence of pulmonic stenosis or severe pulmonary vascular obstructive disease.8 11 Continuing the spectrum from normal to systemic right ventricular pressure, the patients in group III possessed systemic right ventricular pressures with large VSDs. Again, there was a flow of echo-dense material from the right to the left ventricle even though no patient had elevated pulmonary vascular resistance or pulmonary stenosis. Although the time of appearance of contrast in the left ventricle was the same as in group II, these patients were clearly and easily distinguished from those patients in group II by appearance of echo-dense material within the aorta in all group III patients. This never occured in those patients with moderate sized VSDs (group II). Finally, group IV patients exhibited significantly earlier appearance of echo-dense material in the left ventricle than did those patients in group III; Again, all these patients had large VSDs, as did the patients in group III, but the two groups had markedly different hemodynamics. The patients in group IV had obstruction to pulmonary blood flow due to either severe pulmonary stenosis or elevated pulmonary vascular resistance, while those patients in group III had no such obstruction. Thus, patients with large VSDs and right ventricular hypertension could be separated by the flow patterns of echo-dense material into two groups: 1) one group with hyperkinetic pulmonary hypertension and low pulmonary vascular resistance and 2) another group with systemic right ventricular pressure with obstruction to pulmonary blood flow due to either CONTRAST ECHO IN VSD/Serwer et al. 335 Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 FIGURE 7. Recordfrom a patient in Group IVshowing early diastolic appearance of echo-dense material in the L V at the arrow with it remaining in the L V during systole. R V = right ventricle; L V = left ventricle; MV mitral valve. elevated pulmonary vascular resistance or pulmonary stenosis. There were no false positive or negative results in this study. The patterns of intracardiac shunting in the presence of VSDs were first presented by Levin et al.'2 13 Angiographically, they showed this same spectrum - no right-to-left ventricular flow with low right ventricular pressure, transient spilling of blood from the right ventricle to the left ventricle with moderate right ventricular pressure elevation, and, finally, net right-to-left shunting with progressive elevation of the right ventricular pressure. This echocardiographic study demonstrates the same spectrum as their study. The one difference between their study and this study is the timing of the onset of the right ventricular to the left ventricular flow in those patients with moderate and large VSDs. They found it began during isovolumic relaxation, whereas we first observed the shunt just after mitral valve opening. The difference is probably related to the limited area being observed by the echo beam. Two patients, JP and MC, were each studied on two occasions - first in the neonatal period and second near I year of age. Both patients initially showed flow patterns indicative of systemic right ventricular pressure but no obstruction to pulmonary blood flow (group III). At catheterization, both had systemic right ventricular pressure with large left-to-right shunts and normal pulmonary vascular resistance. At restudy, both were doing well clinically. Patient JP still showed the echocardiographic flow pattern of group 1II. Subsequent cardiac catheterization verified the persistence of systemic right ventricular pressure with no pulmonary stenosis and no change in pulmonary resistance, and complete repair was undertaken to avoid the development of elevated pulmonary vascular resistance. However, at restudy MC showed the echocardiographic flow pattern of group II, pointing toward a decrease in the right ventricular pressure. This was confirmed at cardiac catheterization (right ventricular pressure 2/3 systemic pressure), and repair was not felt to be indicated, as there was no risk of elevated pulmonary vascular resistance developing. The patient's subsequent clinical course has supported this decision. Once the validity of contrast echocardiography is established, the need for repeat cardiac catheterization in patients such as MC could be obviated. Conversely, if a patient were to show a change from flow patterns characteristic of group II to those characteristic of group III or group III to group IV, then cardiac catheterization should be considered, as this change would point to a changing hemodynamic state. The patterns presented in this study are not presented primarily as a means of diagnosing structural heart disease, although certain implications concerning structure certainly can be drawn. The major point is that these distinct flow patterns are indicative of specific hemodynamic situations and are extremely valuable in evaluating the hemodynamic status of the patient, particularly on a serial basis. These studies are readily and easily performed in the outpatient setting and provide an accurate means of serially assessing the hemodynamic status in CI RCULATION 336 such patients to determine the best course of future medical management. Acknowledgment Appreciation is expressed to Mrs. Pat Parker for her technical assistance. References Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 1. Gramiak R, Shah PM: Echocardiography of the aortic root. Invest Radiol 3: 356, 1968 2. Gramiak R, Shah PM, Kramer DH: Ultrasound cardiography: contrast studies in anatomy and function. Radiology 92: 939, 1969 3. Feigenbaum H, Stone JM, Lee DA, Nasser WK, Chang S: Identification of ultrasound echoes from the left ventricle by use of intracardiac injections of indocyanine green. Circulation 41: 615, 1970 4. Kerber RE, Kroschos JM, Lauer RM: Use of an ultrasonic contract method in the diagnosis of valvular regurgitation and intracardiac shunts. Am J Cardiol 34: 722, 1974 5. Pieroni D, Varghese PJ, Rowe RD: Echocardiography to detect shunt and valvular incompetence in infants and children. (abstr) Circulation 48 (suppl IV): IV-81, 1973 VOL 58, No 2, AUGUST 1978 6. Seward JB, Tajik AJ, Spangler JG, Ritter DG: Echocardiographic contrast studies. Initial experience. Mayo Clin Proc 50: 163, 1975 7. Seward JF, Tajik AJ, Hagler DJ, Ritter DG: Contrast echocardiography in single or common ventricle. Circulation 55: 513, 1977 8. Valdes-Cruz LM, Pieroni DR, Roland JMA, Varghese PJ: Echocardiographic detection of intracardiac right-to-left shunts following peripheral vein injections. Circulation 54: 558, 1967 9. Valdes-Cruz LM, Pieroni DR, Roland JMA, Shemotek JP: Recognition of residual post-operative shunts by contrast echocardiographic techniques. Circulation 55: 148, 1977 10. Kremkau FW, Gramiak R, Carstensen EL, Shah PM, Kramer DH: Ultrasonic detection of cavitation at catheter tips. AJR 110: 117, 1970 11. Seward JB, Tajik AJ, Hagler DJ, Ritter PG: Peripheral venous contrast echocardiography. Am J Cardiol 39: 202, 1977 12. Levin AR, Spach MS, Canet RV, Boineau JP, Capp MP, Jain V, Barr RC: Intracardiac pressure-flow dynamics in isolated ventricular septal defects. Circulation 35: 430, 1967 13. Levin AR, Boineau JP, Spach MS, Canent RV, Capp MP, Anderson PAW: Ventricular pressure-flow dynamics in tetralogy of Fallot. Circulation 34: 4, 1966 14. Fixler DE, Carrell T, Broune R, Willis K, Miller WM: Oxygen consumption in infants and children during cardiac catheterization under different sedation regimems. Circulation 50: 788, 1974 Two-Dimensional Echocardiographic Assessment of Ebstein's Anomaly THOMAS A. PORTS, M.D., NORMAN H. SILVERMAN, M.D., AND NELSON B. SCHILLER, M.D. SUMMARY Nine patients with Ebstein's anomaly of the tricuspid valve were studied by two-dimensional echocardiography, using the standard long and short axis views as well as the apex four chamber view. With this latter view, the displacement of the tricuspid valve into the right ventricle was clearly seen in all nine cases of Ebstein's anomaly and was not noted in a control population. The severity of the tricuspid displacement was assessed by comparing the position of the mitral and tricuspid valves relative to the cardiac apex. The apex four chamber view allowed visualization of the atrioventricular (AV) ring simultaneously with the displaced tricuspid valve, and therefore the size of the "atrialized" right ventricle, true right ventricle and right atrium could be determined. These dimensions compared favorably with angiography. EBSTEIN'S ANOMALY of the tricuspid valve is a rare congenital heart disorder. It consists of downward displacement of all or part of a malformed tricuspid valve and associated architectural abnormalities of the right ventricle. Recent reviews have stressed the pathologic and clinical spectrum of this disorder.1-3 In the newborn it is frequently accompanied by cyanosis and heart failure, and must be differentiated from other forms of cyanotic heart disease.4 Patients with milder forms may remain asymptomatic, even in adulthood. This varied expression often makes the clinical diagnosis difficult, and a nonFrom the Department of Medicine, Cardiovascular Division, the Department of Pediatrics, and the Cardiovascular Research Institute, University of California, San Francisco, California. Address for reprints: Thomas A. Ports, M.D., Cardiology, 1186Moffitt Hospital, University of California, San Francisco, California 94143. Received January 20, 1978; revision accepted April 16, 1978. invasive means to diagnose and accurately assess the severity of this disorder is important. Phonocardiography, M-mode echocardiography and, more recently, two-dimensional imaging systems have proved useful in the diagnosis of Ebstein's anomaly, but fail to estimate its severity. We evaluated nine patients with Ebstein's anomaly, using a wide-angle (80°) phasedarray sector scanner, using both conventional precordial images and apex echocardiography to define the anatomic severity of this deformity. Materials and Methods Nine patients with Ebstein's anomaly were studied. They ranged in age from 6 hours to 32 years. Clinical severity was variable, ranging from asymptomatic in four to severely symptomatic with cyanosis and/or congestive heart failure in three. Cardiac catheterization and biplane cineangiographic data were available for all patients. The diagnosis of Ebstein's anomaly Use of contrast echocardiography for evaluation of right ventricular hemodynamics in the presence of ventricular septal defects. G A Serwer, B E Armstrong, P A Anderson, D Sherman, D W Benson and S B Edwards Downloaded from http://circ.ahajournals.org/ by guest on June 15, 2017 Circulation. 1978;58:327-336 doi: 10.1161/01.CIR.58.2.327 Circulation is published by the American Heart Association, 7272 Greenville Avenue, Dallas, TX 75231 Copyright © 1978 American Heart Association, Inc. All rights reserved. Print ISSN: 0009-7322. Online ISSN: 1524-4539 The online version of this article, along with updated information and services, is located on the World Wide Web at: http://circ.ahajournals.org/content/58/2/327.citation Permissions: Requests for permissions to reproduce figures, tables, or portions of articles originally published in Circulation can be obtained via RightsLink, a service of the Copyright Clearance Center, not the Editorial Office. Once the online version of the published article for which permission is being requested is located, click Request Permissions in the middle column of the Web page under Services. Further information about this process is available in the Permissions and Rights Question and Answer document. Reprints: Information about reprints can be found online at: http://www.lww.com/reprints Subscriptions: Information about subscribing to Circulation is online at: http://circ.ahajournals.org//subscriptions/