March 15, 1984 lliE AMERICAN JOURNAL OF CARDIOLOGY Volume 53 971

Bedside Balloon Atrial Septostomy Using Echocarldiographic Monitoring

CHARLES A. BULLABOY, MD RUFUS B. JENNINGS, Jr., MD

DAVID H. JOHNSON, MD, With the technical assistance of CAROL W. FULCHER, RDMS

Two-dimensional echocardiography (2-D echo) has recently been used as an adjunct to balloon atria1 sep- to&my (BAS) in infants with cyanotic congenital heart disease.ls2 However, this technique is not routinely used in place of cardiac catheterization. We have performed 2-D echo-guided BAS in 3 newborn infants without simultaneous cardiac catheterization or fluoroscopy.

In May 1980, an infant weighing 2.7 kg was transferred to our hospital at 19 hours of age. He was in shock, with active bleeding and laboratory evidence for disseminated intra- vascular coagulation. His hydrogen ion concentration was 6.7 with an oxygen tens&yn (PO2) of 24 mm Hg. Two-dimen- sional echo revealed transposition of the great vessels with intact ventricular septum. Cardiac catheterization confirmed this diagnosis and BAS was performed with fluoroscopic control. No improuemeni in oxygenation occurred after BAS, and repeat 2-D echo demonstrated an atria1 septal defect of only 15% of the septal diameter. Monitored only by 2-D echo, repeat BAS was then carried out in the neonatal intensive care unit, using the subniphoid 4-chamber view to visualize the catheter in the inferior vena cava, right atrium and left atrium. Repeat 2-D echo then demonstrated an atria1 septal defect of 25% of the septal diameter. The infant remained hypoxic and subsequently died from severe metabolic prob- lems. Autopsy demonstrated a torn foramen membrane with a defect measuring 10 x 5 mm in the fixed state. This case represented our first experience with 2-D echocardiographic monitoring of BAS.

A twin weighing 1.04 kg was delivered with breech pre- sentation and required intubation and positive pressure ventilation in the delivery room. His PO2 did not improve on 100% oxygen. Two-dimensional echo demonstrated trans- position of the great ves.sels with intact ventricular septum. BAS was then performec! in the neonatal unit monitored only

From the Department of Pediatrics, Division of Pediatric Cardiology, Children’s Hospital of the King’s Daughters, Eastern Virginia Medical School, 800 West Olney Road, Norfolk, Virginia 23507. Manuscript received October 3, 1983; revised manuscript received November 5, 1983, accepted November 9, 1983.

by 2-D echo with an immediate increase in arterial PO2 from 21 to 37 mm Hg. The infant subsequently died from multiple problems. At autopsy a 0.8-cm rent was present in the fora- men ovale membrane.

A l-day-old, 3.1-kg term infant was transferred to our hospital because of severe cyanosis. Two-dimensional echo showed transposition of the great vessels with intact ven- tricular septum. Because the cardiac catheterization labo- ratory was not available to us for several hours, we performed BAS using 2-D echo monitoring in the neonatal intensive care unit. After BAS the patient remained dependent on prostaglandins to maintain an adequate POa. A Blalock- Hanlon procedure was performed, with no subsequent im- provement in oxygenation. A cardiac catheterization con- firmed the diagnosis of transposition and failed to clarify the etiology of the continuing hypoxemia. A Mustard procedure was then performed at 3 weeks of age with an excellent postoperative course. At surgery a large BAS septal defect was present, as well as a large Blalock-Hanlon defect; the 2 defects were separated by a thin strand of tissue.

In these cases BAS monitored only by 2-D echo ap- peared to be the most feasible option under difficult circumstances-the nonavailability of the cardiac catheterization laboratory and the difficulties inherent in transporting intubated and hemodynamically un- stable infants. Two-dimensional echocardiographic diagnosis of newborn infants with transposition of the great arteries is safely and accurately performed in the neonatal intensive care unit.3 Previous reports have demonstrated the value of ultrasound for monitoring BAS,~T~ although in previously reported cases 2-D echo was used as an adjunct to fluoroscopy in the cardiac catheterization laboratory. However, 2-D echo is su- perior to fluoroscopy in defining the relations between the septostomy catheter and the atria1 septum, the pulmonary veins and the atrioventricular valves. As experience with this technique increases, we believe that 2-D echo monitoring should replace standard cardiac catheterization for BAS in selected cases.

Acknowledgment: We express our appreciation to Brenda Taylor for her assistance in preparation of this manuscript.

References

1. Perry LW, Ruckman RN, Galloto FM Jr, Shapiro SR, Potter BM, Scott LP. Echocardqraphically assisted balloon atrial septostomy. Pediatrics 1982;70:403-408.

2. Allan LD, Leanage R, Wainwright R, Joseph MC, Tynam M. Balloon atrial septostomy under two-dimensional echocardiographic control. Br Heart J 1982;47:41-43.

3. Bierman FZ, Williams RG. Prospective diagnosis of d-transposition of the eat arteries in neonates by subxiphoid two-dimensional echocardiography.

F irculabon 1979:60:1496-1502.

Quadricuspid Pulmonic Valve WILLIAM LEWIS, MD

CARMINE CAMMAROSANO, BS

From the Department of Patihology’, UCLA Medical Center, Loa Angeles, California 90024. Manuscript received October 17, 1983; revised manuscript received October 28, 1983, accepted October 31, 1983.

Congenitally quadricuspid pulmonic valve has been described,lT2 but has not received the attention that the more common bicuspid aortic valve has received, probably because of the clinical silence of the former lesion. The incidence of quadricuspid pulmonic valve varies from 0.02 to 0.12%; approximately 250 cases have been reported.lT2 The variation in the reported incidence of this lesion may relate to methods of autopsy study because minor abnormalities of semilunar valves may be easily overlooked. A necropsy was performed on a

972 BRIEF REPORTS

64-year-old man who died of chronic renal failure. At autopsy, 4 cusps were found on the puhnonic valve (Fig. 1). All pulmonic cusps were thin and delicate. Three cusps were of normal size and the fourth cusp was ru-

FIGURE 1. Necropsy photograph shows right ventricular outflow with quadricuspid pulmonic valve with 3 normal cusps and 1 rudimentary cusp (arrow). Commissures of quadricuspid valve are distinct.

dimentary. Review at UCLA Medical Center disclosed, among 10,384 autopsies over 28 years (1955 to 1982), 10 additional cases. Of these, 7 were adults, aged 37 to 71 years, and the condition was discovered incidentally at necropsy. The 3 other patients were aged 1 to 12 months; 1 had ventricular septal defect and patent ductus arteriosus. The incidence of quadricuspid pul- manic valve is 0.11% at UCLA. In cases in which the valve abnormality was described, 1 rudimentary cusp and 3 normal cusps were reported. No patient had symptoms related to the pulmonic valve. Quadricuspid pulmonic valve is a clinically quiescent anatomic anomaly.

References 1. Hurwlir I., Roberts WC. Quadricuspid semilunar valve. Am J Cardlol

1973;31:623-626. 2. Davla JE, Fenogllo JJ, Decasiro CM, McCaltlster Jr HA, Che~ltlln MD.

Quadricuspid semilunar valves. Chest 1977;72:166-169.

Diagnosis of Incompetent Quadricuspid Aortic Valve By Two-Dimensional

Echocardiography

ROBERT L. HERMAN, MD IRA S. COHEN, MD

KENNETH GLASER, MD EVERETT W. NEWCOMB III, DO

Quadricuspid aortic valve is a rare developmental anomaly often associated with aortic regurgitation

From the Cardiology Section, Department of Medicine, Walter Reed Army Medical Center, Washington, DC. 20307. Manuscript received October 21, 1983; revised manuscript received November 10, 1983, accepted November 11, 1983.

The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of Defense or the Departments of the Air Force or Army.

(AR).l Since 1923,16 quadricuspid aortic valves have been noted at autopsy, at surgery, or during aortography for AR. We report the first case of a quadricuspid aortic valve detected noninvasively by 2-dimensional echo- cardiography (2-D echo).

A 59-year-old woman was referred for evaluation of an AR murmur, Two-dimensional echo was performed using a Di- asonics 3400R ultrasonograph. A diastolic frame with the approximately equal-sized cusps meeting at a central rec- tangular coaptation point is shown in Figure 1 (left) and a systolic frame in Figure 1 (right). Each cusp moved inde- pendently without evidence of incomplete separation as a raphe, as can be seen in bicuspid aortic valves.

The 2-D echo demonstration of a quadricuspid aortic valve widens the spectrum of hemodynamically signif- icant aortic valve diseases that can be diagnosed by this means.

Reference

1. Hurwiiz LE, Roberts WC. Quadricuspid semilunar valve. Am J Cardiol 1973;31:623-626.

FIGURE 1. Left, short-axis view of the aortk valve in dtastole. A “4-cuspid- clover” appearance replaces the nor- mal “Y” configuration. OT = right ventricular cutfkw tract; RA = right atrium: LA = left atrium. Right, short- axis view in systole. The bfack and white arrows indicate each cusp.