Right heart catheterization in patent ductus arteriosus and aortic-pulmonary septal defect

R I G H T H E A R T C A T H E T E R I Z A T I O N IN P A T E N T DUCTUS ARTERIOSUS AND AORTIC-PULMONARY S E P T A L D E F E C T FORREST

H.

ADAMS,

M.D., A_~TONI D I E H L ~ MID., Jos]~PH J O R G E N S , L. GEORGEVEASY, M.D. ~ MINNEAPOLIS~

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pulmonary septal defect are the subject of this r e p o r t . 9 Eight of the ductus arteriosus are well recognized. 1-5 twenty-four patients were considered, That not all patients with patent duc- before the studies were made, to have tus arteriosus have the typical clinical the typical findings of patent ductus findings is well documented. 2 Since arteriosus by physical, roentgen, and the poor prognosis of untreated patent electrocardiographic examination. Sixductus arteriosus and the good results teen patients had atypical clinical findof surgical intervention are fairly ings either by physical or roentgen well-established facts, 1, 2, 5-s it is im- examination or by electrocardiography. portant that all patients with this con- Of this latter group after the studies dition be recognized. were completed, six were interpreted Physiological studies on patients to have an aortic-pulmonary septal dewith patent ductus arteriosus have been fectP reported on several occasions2, o-16 One of the patients diagnosed by Such studies are not only of scientific heart catheterization as having an value in order that more knowledge aortic-pulmonary septal defect was can be gained concerning the pathoproved by post-mortem examination. logical physiology involved, but partiWe are aware of no previous instance cularly with the advent of cardiac in which the diagnosis of this defect catheterization, a new tool has been was made ante mortem. In both the added that can aid in the diagnosis of typical and atypical group of patients the cardiac anomaly when the p a t i e n t with patent duetus arteriosus the diagpresents atypical findings. The purnosis was confirmed at surgery in each pose of this communication is to indicase. cate the value of cardiac catheterizaRESULTS 9tion in such atypical cases and to corTypical Patent Ductus Arteriosus.-relate the degree of the pathological physiology involved with clinical symp- TaMe I shows the clinical data on the eight patients considered to have a tomatology. Twenty-four patients with either a ~ypical patent ductus arteriosus. Table patent ductus arteriosus or an aortic- II contains the physiological data of these eight patients obtained by right From the Departments of Pediatrics and Radiology, University of Minnesota. heart catheterization. In two of them This work was performed under a cooperative agreement between the University of the catheter was passed through the symptoms and clinical findings T inH E patients with typical patent

/~IinnesOta a n d t h e N a t i o n a l H e a r t I n s t i t u t e , U. S. P u b l i c H e a l t h S e r v i c e , ] ~ e t h e s d a , IcId. *American Heart Association Research Fellow. **Alpha Phi Heart Research Fellow.

* O n e of t h e s i x p a t i e n t s i n t e r p r e t e d a s having an aortic-pulmonary septal defect was proved at surgery rather to have an enormous patent ductus arteriosus. 49

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Fig. L - - P a t i e n t B. ]~., 1 5 - y e a r - o l d f e m a l e w i t h a t y p i c a l p a t e n t d u c t u s a r t e r i o s u s , l:toentgenograms ~ and b show enlarged heart, prominent pulmonary artery segment, enlarged root s h a d o w s , a n d prena~nent p u l m o n a r y v a s c u l a r m a r k i n g s . Intracardiac pressure recordings v and d show pulmonary artery and right ventricular hypertension as catheter was withdrawn t h r o u g h t h e p u h n e n a r y v a l v e . P h o n o c a r d i o g r a m s t a k e n in s e c o n d l e f t ( v ) a n d t h i r d l e f t ( f ) i n t e r s p a c e c o n f i r m p r e s e n c e of i s o l a t e d s y s t o l i c m u r m u r .

A D A M S E T AL. :

P A T E N T DUCTUS ARTERIOSUS

duetus arteriosus into the descending aorta, thus proving the diagnosis. Two of this group of patients, M.P. and J.P.,O had right ventricular and pulmonary artery pressures that were elevated above normal (30 ram. of mercury, systolic). The left-to-right shunt through the ductus varied from 0.5 to 15.2 liters per minute. This represented from 7 to 66 per cent of the left ventricular output. As can be seen in Table II the amount of shunt did not correlate too well with the symptomatology; that is, some. patients with large shunts had relatively few symptoms.

Atypical Patent Ductus.--Table I I I shows the clinical data on the ten patients other than those with aorticpulmonary septal defects who were considered clinically not to have a typical patent ductus arteriosus. Two o~ these patients had only a systolic murmur audible over the left precordium and were thought clinically to have an interatrial defect. Several other patients had atypical diastolic murmurs. Six patients had abnormal electrocardiograms, three with right axis deviation. Six patients had roentgen findings not consistent with a typical patent duetus arteriosus. Cardiac catheterization clearly demonstrated that the left-to-right shunt existed between the aorta and the pulmonary artery rather than between the .atria as seen in Table IV. In three instances the catheter was passed through the duetus arteriosus thus proving the diagnosis. The phonocardiogram recordings, intracardiae * T h e e x a c t p r e s s u r e s o b t a i n e d in p a t i e n t ft. P. w e r e n o t r e c o r d e d in t h e t a b l e b e c a u s e of t e c h n i c a l difficulties in s t a n d a r d i z a t i o n o f the instrument.

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pressure curves, and roentgen findings on patient B.B. can be seen in Fig. 1. From Table IV it can be seen that the shunts varied from 0.8 to 11.1 liters per minute; this represented from 24 to 67 per cent of the left ventricular output. (Two patients had higher shunts when expressed as percentage of ]eft ventricular output, but the absolute values were not obtained.) Seven of these ten patients had mild to moderate cardiac disability.

Aortic-Pulmonary Septal Defect.-Table V shows the clinical data on six .patients who following these studies were interpreted to have an aorticpulmonary communication or defect near the base of the heart. This diagnosis was based on the location of the murmur and the presence os a left-toright shunt in the region of the pulmonary artery. Only one of these patients had a machinery murmurS"; the other patients bad a systolic murmur either alone or in combination with a diastolic murmur best heard in the third and fourth left interspace. Five of the six patients had abnormal electrocardiogr.ams consisting of right axis deviation in two and large biphasic QRS complexes in four. Three of the six patients were thought by roentgen examination to have an interatrial defect combined with mitral stenosis. As seen in Table VI, all of the patients had a right ventrieular hypertension and pulmonary artery hypertension except M.K. The shunts varied from 4.4 to 21.9 liters per minute, and the shunt expressed as per cent of the *A p a t e n t ductus a r t e r i o s u s w a s proved n o t to b e p r e s e n t a t s u r g i c a l e x o l o r a t i o n a n d the surgeons described the m u r m u r a n d thrill a s o r i g i n a t i n g a t t h e b a s e of t h e h e a r t . 1~0 a t t e m p t w a s m a d e to d i s s e c t o u t t h i s p a t i e n t ' s defect.

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P A T E N T D U C T U S ARTEI%IOSUS

left ventrieular o u t p u t varied f r o m 46 to 85 per cent. F o u r of the six patients had mild to moderate cardiac disability. I n one patient, J. II., the catheter was passed through the defect, into the aorta .as seen in Fig. 2, thus proving the diagnosis. The anterior position of the catheter in the lateral view showed that it had not passed through the duetus arteriosus.

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feet, and also the roentgen findings in this ease. Although following the catheterization studies patient D. S. was thought to have an aortic-pulmonary septal defect primarily because of the location of his heart murmurs, this proved not to be the case. At surgical exploration an enormous patent duetus arteriosus was found and there was no defect in the adrtic-pulmonary septum region.

l~ig. 2 . - - P a t i e n t J. I-I., 6 - y e a r - o l d m a l e w i t h a o r t i c - p u l m o n a r y s e p t a l d e f e c t . Roentgeno~ g r a m s a a n d b s h o w p o s i t i o n of c a t h e t e r w h i c h h a s p a s s e d f r o m t h e pulraonar:~" a r t e r y t h r o u g h t h e d e f e a t i n t o t h e a s c e n d i n g a o r t a . T h e tip is n e a r t h e a o r t i c v a l v e s . N o t e h o w t h e c a t h e t e r r e m a i n s a n t e r i o r in t h e l a t e r a l v i e w t h u s s h o w i n g it h a d n o t p a s s e d i n t o a p a t e n t d u e t u s arteriosus a n d then into the aorta.

Patient K. N. was suspected clinically of having an aortic-pulmonary septal defect, because of the location of ~l~e murmurs, before tile heart catheterization studies were made, and as can be seen in Table VI, these studies showed the shunt to be in the region of the pulmonary artery. A surgical attempt to close the defect in this patient was unsuccessful, and she expired on t h e operating table. Fig. 3 shows the heart specimen illustrating the de-

Following ligation of the ductus, however, there remained a thrill in the region of the interventrieular septmn, and postoperatively there was clinical evidence that he had a residual interventrieular septal defect.

Relationship of Shunt tv D~s~bilitg c~nd Pulmonary Artery Pressure.-Fig. 4 shows the shunt ~s per cent of the left ventrieular output plotted against the disability in the patients studied. It can be seen that there was

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a number of patients that had rather large shunts but no cardiac disability. Those patients with mild to moderate cardiac disability (with one exception}, all had sizeable shunts.

0F

PEDIATRICS

Fig. 5 shows the shunt as per cent of the left ventrieu]ar output plotted against the pulmonary artery pressure. Very large shunts were present in some patients without producing pulmonary

Fig. 3.--Patient I4:. N., 13-year-old female with aortic-pulmonary septal defect, l~oentgenograms a and b show enlarged, heart, prominent pulmonary artery segment, and prominent peripheral vessels. Heart speclmen e shows defect at base of heart between aorta and pulmonary artery. Arrow shows direction of blood flow out the aorta and also through the defect into the Dulmonary artery,

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hypertension. All patients except one with pulmonary hypertension had large shunts. Fig. 6 shows the cardiac disability plotted against the pulmonary artery pressure. It can be noted that alI the patients who had no disability had relatively normal pressures, whereas most of those with disability had pulmonary hypertension. DISCUSSION

Although right heart catheterization is of questionable clinical value in a patient who has the typical clinical ~ndings of patent ductus arteriosus, it is of definite value in the atypical ease. In those patients in whom the murmur, or the elctrocardiogram, or the roentgenograms were atypical, the data obtained by right h e a r t catheterization were extremely illuminating. Several patients thought to have some other defect were found by this technique to have a patent ductus arteriosus, and thus too received the benefit of surgical ]igation. In several instances the diagnosis was proved before surgery by passing the catheter through the dnctus arteriosus and down into the aorta. 1~ Using the heart catheterization technique our observations on pulmonary blood flow and the amount of shunt agree quite well with those of previous investigators.i, 9, 16 Taylor and associates 16 found no consistent relationship between the size of the duetus arteriosus and the amount of shunt through it. In our studies, although some patients had large shunts with normal pulmonary artery pressures, most of the patients with elevated pressures had large shunts. Increasing cardiac disability seemed to be related to the increase in pulmonary artery pressure.

Right heart catheterization is also of value in establishing the diagnosis' of an aortic-pulmonary septal defect. In some instances it is possible to pass the catheter through the defect into the aorta, thus proving the diagnosis. This can be distinguished from the situation where the catheter has passed through the duetus arteriosus into the aorta by the anterior position of the catheter in the lateral view. In other instances the diagnosis can only be presumed in those patients who have a left-to-right shunt in the region of the pulmonary artery as shown by heart catheterization and who on physiaal examination have a murmur with the maximum intensity in the .region of the third and fourth left interspace or just beneath the sternum. According to Spencer, 17 only thirteen instances of aortic-pulmonary defect have been previously reported in the literature up to 1950. Ten of the cases were from Maude Abbott's collection. Spencer added an additional case of his own in his review. Since we have collected five patients in less than two years as a result of these physiological studies, it seems probable that this defect is not as rare as is indicated from the literature. Recently Gibson and co-workersis have reported four patients operated on for patent ductus arteriosus in whom no ductus was found. The murmur and thrill were described by the surgeon as coming from the base of the heart. This suggested to the authors that the patients probably had an aortic-pulmonary defect. The clinical impression was not corroborated by physiological data or post-mortem examination, however. Only one of our patients with aortic-pulmonary defects

ADAMS ET AL. :

PATENT DUCTUS ARTERIOSUS

was thought clinically to have a patent duetus arteriosus before these studies were made. SUM!VIARY

1. Right heart catheterization studies on eight patients with typical patent ductus arteriosus, on eleven patients with atypical patent ductus arteriosus, and on five patients with an aorticpulmonary defect have been presented. 2. The importance and the value of this technique as an aid to the diagnosis in the patient with atypical clinical f n d i n g s has been discussed. 3. Data were presented to show that the cardiac disability was related more to the pulmonary a r t e r y pressure than to the size of the shunt. 4. Data on five patients with aorticpulmonary defects were presented. One patient expired and at post-mortem examination, the diagnosis was confirmed. This is the first instance of such a diagnosis being m a d e ante mortem and confirmed at autopsy reported thus far in the literature. REFERENCES I. Shapiro, IVL J., Keys, A. : Clinical and Physiologic Analysis of Twenty-Three Patients With Persistent Patent Ductus Arteriosu% Internat. Clin. 4: 149, 1941. 2. Keys, A., and Shapiro, IVL J.: Patency of Duetus Arteriosus in Adults, Am. H e a r t J. 25: 158, 1943. 3. Shapiro, 1VI. J. : The Preoperative Diagnosis of P a t e n t Duetus Arteriosus, J. A. 1V[. A. 126: 934, 1944. 4. Levine, S. A. : The Auscultatory Findings in P a t e n t Ductus Arteriosus, Bull. New Englaud It[. Center 8: 148, 1946. 5. Benn, J . : The Prognosis of Patent Ductus Arteriosus, Brit. Heart. J. 9: 283, 1947.

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6. Gross, 1%. E., and Hubbard, J. P. : Surgi eal Ligation of a P a t e n t Ductus Arteriosus" Report of First Successful Case, J. A. 1~. A. 112: 729, 1939. 7. Shapiro, IV[. J., and Keys, A. : The Prognosis of Untreated P a t e n t Ductus Arteriosus and the Results of Surgical Intervention, Am. $. M. So. 206: 174, 1943. 8. Scott, IX. W. : Closure of the P a t e n t Ductus by Suture-Ligation Technique, Surg., Gynee. & Obst. 90: 91, 1950. 9. Eppinger, E. C., Burwell, C. S., and Gross, 1%. E.: The Effects of the P a t e n t Ductus Arteriosus on the Circulation~ J. Clin. Investigation 20: 127, 1941. 10. Dexter, L., Haynes, F. W., Burwell, C. S.~ Eppinger, E. O., Sosman, M. C., and Evans, J. IV[.: Studies of Congenital H e a r t Disease. III. Venous Catheteriza tion as a Diag~aostie Aid in P a t e n t Ductus Arteriosus, Tetralogy of Fallot, Ventrieular Septal Defect, and Auricular Septal Defect, J. Clin. Investigation 26: 561, 1947. 11. Vandam, L. D., Bing, 1%. J., and Gray, F. D., Jr. : Physiological Studies iu Congenital I~eart Disease, Bull. Johns Hopkins Hosp. 81: 192, 1947. 12. Cournand, A., Baldwin, J. S., and I-Iimmetstein, A.: Cardiac Catheterization in Congenital H e a r t Disease, New York, 1949, The Commonwealth Fund. 13. Smith, K. S., and Wood, F. G.: Radiokymography in P a t e n t Ductus Arteriosus, Brit. Heart J. 11: 257, 1949. 14. Adams, F. IX., LaBree, J. W., and Stauffer, H. M.: Right H e a r t Catheterization in Acyanotic Congenital H e a r t Disease, Journal-Lancet 70: 159, 1950. 15. Adams, F. H., LaBree, 5. W., and Stauffer, H. IV[.: Right l