248
Brief Communications
Transesophageal echocardiography was extremely useful in following this patient postoperatively as well. On the seventh postoperative day transesophageal echocardiography revealed recurrence of vegetations on both mitral leaflets with abscess formation on the anterior mitral leaflet. Daniel et al.‘O found that transesophageal echocardiography was much more sensitive than transthoracic echocardiography in detecting abscess consequent upon endocarditis. After the detection of these abscesses and significant mitral regurgitation, the patient was taken for mitral valve replacement. The intraoperative transesophageal echocardiogram revealed that some of the multiple abscess spaces had ruptured to form a sinus tract through the anterior mitral leaflet. Recognition of pacemaker lead infection can be difficult. Transthoracic echocardiography is helpful, but image resolution may be inadequate in patients with small acoustic windows. While there are no large studies comparing the sensitivity of transthoracic echocardiography to transesophageal echocardiography in detecting pacemaker lead vegetations, this example, as well as the case reports by Van Camp et al.‘l and Zehender et a1.12 illustrates the detection of pacemaker lead vegetations by transesophageal echocardiography that were not visualized with transthoracic echocardiography. While transesophageal echocardiography offers significant advantages over transthoracic echocardiography in selected cases, it still has limitations in sensitivity, as in this case in which the vegetations on the tricuspid valve chordae and the right ventricular portion of the pacemaker lead were not visualized by transesophageal echocardiography but were subsequently found at surgery. The sensitivity of transesophageal echocardiography appears to be somewhat dependent on the number of views and planes in which the various structures are visualized. Transthoracic echocardiography will remain the standard of care when evaluating valvular competence and left ventricular function in patients with endocarditis having satisfactory acoustic windows. However, transesophageal echocardiography, because of its superior resolution and hence higher sensitivity in the detection of vegetations, should be considered as a valuable adjunct, especially in patients with poor acoustic windows or when complications are suspected. Serial examinations are also useful when surgery is planned or clinical deterioration occurs. REFERENCES
1. Yoshida K, Yoshikawa J, Yamaura Y, Hozumi T, Akasaka T, Fukaya T. Assessment of mitral regurgitation by biplane transesophageal color Doppler flow mapping. Circulation 1990;82:1121-6. 2. Lerner PI, Weinstein L. Infective endocarditis in the antibiotic era. N Engl J Med 1966;274:199-206. 3. Jung JY, Saab SB, Almond CH. The case for early surgical treatment of left-sided primary infective endocarditis. d Thorat Cardiovasc Surg 1975;70:509-18. 4. Jaffe WM, Morgan DE, Pearlman AS, Otto CM. Infective endocarditis, 1983-1988: echocardiographic findings and factors influencing morbidity and mortality. J Am Co11 Cardiol 1990;15:1227-33. 5. Martin RP. The diagnostic and prognostic role of cardiovas-
July 1992 Hean Journal
American
6.
7.
8.
9.
cular ultrasound in endocarditis: bigger is not hetter. .J .4m Co11 Cardiol 1990;15:1234-7. Stewart JA, Silimperi D, Harris P, Wise NK, Fraker TD, Kisslo JA. Echocardiographic documentation of vegetative lesions in infective endocarditis: clinical implications. Circulation 1980;61:375-80. Mugge A, Daniel WG, Frank G, Lichtlen PR. Echocardiography of infective endocarditis: reassessment of prognostic implications of vegetation size determined by the transthoracic and the transesophageal approach. .J Am Co11 Cardiol 1989;14:631-8. Erbel R, Rohmann S, Drexler M, Mohr-Kahaly S, Gerharz CD, Iversen S, Oelert H, Mever +J. Improved diagnostic value of echocardiography in patients with infective endocarditis by transesophageal approach. A prospective study. Eur Heart .J 1988;8:43-53. Taams MA, Gussenhoven EJ, Bos E, de daegere P, Roelandt .JRTC. Sutherland GR. Born N. Enhanced moroholonical diagnosis in infective endocarditis by transesophageal echocardiography. Br Heart J 1990;63:109-13. Daniel WG, Mugge A, Martin RP, Lindert 0, Hausmann I), Nonnast-Daniel B, Laas J, Lichtlen PR. Improvement in the diagnosis of abscesses associated with endocarditis by transesophageal echocardiography. N Engl J Med 1991:324:795-800. Van Camp G, Vandenbossche JL. Recognition of pacemaker lead infection by transesophageal echocardiography. Br Heart J 1990;65:229-30. Zehender M, Buchner C, Geibel A, Kasper W, Meinertz T, Just H. Diagnosis of hidden pacemaker lead sepsis by transesophageal echocardiography and a new technique for lead extraction. AM HEART J 1989;118:1050-3. I
10.
11.
12.
”
Congenital isolated pulmonary valve incompetence: Neonatal presentation and early natural history William Berman Jr., MD, Raymond R. Fripp, MD, Stuart A. Rowe, MD, and Steven M. Yabek, MD. Albuquerque, N.M.
Congenital dysplasia of the pulmonary valve leaflets, unassociated with a ventricular septal defect, is a rare congenital anomaly, having been described during life in about two dozen patients, only four of whom were neonates at the time of diagnosis. i, 2 We summarize our clinical and hemodynamic observations on two extensively studied neonates to define more clearly the presentation, management, and early natural history of this lesion. Case No. 1. I.G. weighed 3.9 kg. at birth, was cyanotic in room air, had an increase in respiratory effort, an active precordium, and a grade 416 to-and-fro systolic and diastolic murmur. Chest x-ray films showed marked cardiomegaly, and the electrocardiogram (ECG) showed severe right
From The University Pediatrics. Reprint requests: William of Pediatrics, UNMH-4N. 4f4137234
of New
Mexico
School
Berman Jr.. MD, Albuquerque,
of Medicine, UNM School NM 87131.
Department of Medicine,
of Dept.
Volume Number
124 1
Brief Communications
249
Fig. 1. A, Two-dimensional, short-axis echocardiographic view of right ventricular outflow tract, the pulmonary valve anulus, and the distal main pulmonary artery segment. B, Two-dimensional apical image demonstrating the relative sizes of the right ventricle and left ventricle. C, Two-dimensional short-axis image illustrating the size discrepancy between the main pulmonary and right branch pulmonary artery. Ao, Aortic root; Lt. left; LV, left ventricle; MPA, main pulmonary artery; MV, mitral valve; PAn, pulmonary artery; Rt, right; RV, right ventricle; RVOT, right ventricular outflow valve anulus; RPA, right pulmonary tract; TV, tricuspid valve; VS, ventricular septum. Table
I. Cardiac
catheterization Hb
Age
Hb, Hemoglobin; ovale.
R V pressure
fgmldli
Case No. 1 16 hr 1 Yr 2.8 yr 5.1 yr Case No. 2 1 day RV, right
data PA pressure
Systemic
(mmHd
(mmHd
pressure
(mmHg)
18 13 13 12
80/14 3615 3216 4416
63/30 no/10 22/10 24112
LV Ao Ao Ao
16
4814
35/10
LV = 5515
ventricle;
PA, pulmonary
artery;
Ao, aortic;
LV,
ventricular hypertrophy (RVH). An echocardiogram confirmed the presence of RVH, showed right ventricular cavity dilatation, a large main pulmonary artery, and abnormal appearing pulmonary valve leaflets with a minimally narrowed pulmonary valve anulus. No ventricular septal defects (VSD) was seen. Cardiac catheterization showed a restrictive patent ductus arteriosus (PDA), a small systolic pressure gradient across the pulmonary anulus, and mod-
= = = =
left ventricular;
Associated
72115 85160 105/60 112/81
PDA,
patent
lesions
Small None None None
PDA,
It to rt f.o. shunt
Small
PDA,
bidirectional
ductus
arteriosus;
It, left;
atria1 rt, right;
shunt
f.o., foramen
erately severe pulmonary valve incompetence (Table I). A loud systolic ejection click developed, and at 1 year of age repeat cardiac catheterization showed closure of the ductus arteriosus, a 16 mm Hg systolic pressure gradient across the pulmonic anulus, and mild pulmonary valve incompetence (Table I). The patient was recatheterized at age 3 years because of persistent cardiomegaly and increasing main pulmonary artery dilatation. The hemodynamics
250
Brief Communications
American
Heart
July 1992 Journal
Fig. 2. Color and continuous wave Doppler depictions of right ventricular outflow tract hemodynamics. A, View shows systolic turbulence beyond the level of the pulmonary valve anulus during systole. B, There is a broad diastolic incompetent jet through the valve anulus into the right ventricular outflow tract. C, A continuous wave Doppler tracing from the main pulmonary artery shows modest velocities of outflow turbulence (beneath the baseline) and pulmonary valve incompetence (above the baseline). m/s, meters/second.
were similar to those 2 years before (Table I). At 5 years of age, echocardiographic evidence of increasing pulmonary valve incompetence and right ventricular dilatation prompted the fourth cardiac catheterization. The systolic pressure gradient across the right ventricular outflow tract was slightly higher and the pulmonary valve incompetence was graded as moderate, but the tricuspid valve was competent and clearance of contrast from the right ventricle after angiography was brisk (Table I). The patient was last seen at 8% years of age and was asymptomatic. Case No. 2. A.G. weighed 3.1 kg at birth, was cyanotic but in little respiratory distress, and had an active precordium with systolic and diastolic thrills and a grade 4/6 to-and-fro systolic and diastolic murmur. A chest x-ray film showed massive main pulmonary artery segment dilatation; an ECG showed right ventricular hypertrophy. The echocardiogram showed massive right ventricular dilatation with dysplastic pulmonary valve leaflets and a 26 mm diameter
distal main pulmonary artery, but a 9 mm diameter pulmonary anulus (Fig. 1) and severe pulmonary valve incompetence (Fig. 2). The ventricular septum was intact. Cardiac catheterization showed massive right ventricular enlargement and main pulmonary artery dilatation (Fig. 3), a tortuous patent ductus arteriosus, trivial pulmonary annular stenosis, and severe pulmonary valve incompetence (Table I). Oxygen was administered to promote the physiologic fall in pulmonary vascular resistance. The patient showed no respiratory or cardiac distress over the first 3 months of life. Although cardiac physical findings currently are unchanged, the patient is asymptomatic and has nearly doubled his birth weight in 34’2 months. The term “absent pulmonary valve” in fact refers to a condition in which the pulmonary valve leaflets are rudimentary or dysplastic, the valve anulus is narrowed, and the hemodynamic consequence is mild right ventricular outflow tract obstruction and some degree of pulmonary
Volume
124
Number
1
valve incompetence. Some reports of congenital pulmonary valve absence refer to the syndrome in which pulmonary valve dysplasia coexists with a ventricular septal defect. This condition is referred to often as “tetralogy of Fallot with absent pulmonarv valve” and frequently is associated with neonatal respiral ory distress caused by airway compression hy aneurysmally dilated branch pulmonary art,erie$.” Little is known of the natural history of infants with isolated, congenital pulmonary valve “absence.” We can find only seven case reports of infants with absent pulmonar\’ valve and intact vent.ricular septum. The course and management of these infants have varied widely. Four died shorrly after birth (two of them during surgery), two underwent prosthetic valve insertion, and one had a large PDA ligated.a-“’ The two neonates we describe showed cyanosis but minimal respiratory distress and were managed conservatively because pulmonary valve incompetence (not stenosis) was the dominant lesion and the branch pulmonary arteries were not large enough to cause compression of the airways. In the presence of severe valvar regurgitalion, any attempt 1o reduce the systolic pressure gradient across the right ventricular outflow tract seemed unwise; because insertion of a prosthetic device is problematic in a neonate, and because airway obstruction was not severe, we treated the patients expect,antly. Severe respiratory distress did not develop, cyanosis and dyspnea resolved with time. and both patients are doing well without surgical intervention. At least three developmental processes are likely to promote hemodynamic improvemen: of this lesion with time. As the ductus arteriosus narrows and closes postnatally, the fetal aortopulmonary connection is eliminated. Consequently, both pulmonary flow and pressure will fall, reducing two of the factors promoting pulmonary valve incompetence. Second, with time and ventilation, pulmonary arteriolar resistance falls as a consequence of both remadelling and growth of the alveolar/ arteriolar units. This in turn permits pulmonary artery diastolic pressure to fall, reducing the driving pressure for pulmonary valve incompetence. Third, an independent and close relationship exists between hematocrit and calculated pulmonary vascular resistance.l’ With the postnatal, physiologic fall in hematocrit, a progressive reduction in pulmonary arteriolar resistance and pressure would be expected. The long-term outlook for these patients must be guarded. Intercurrent conditions that elevate pulmonary artery pressure can inr*rease t,he degree of pulmonary valve incompetence and precipitate right heart failure. Also, pulmonary valve incompetence may increase with time and cause tricuspid valve incompetence with eventual right heart failure. However, because physiologic, age-related factt:lrs promote improvement in infants with isolated, congenital “absence” of the pulmonary valve, we recommend conservative management unless respiratory distress makes surgical intervention unavoidable.
Brief Communications
Fig. 3. Still frame from a right ventricular angiogram performed in case No. 2 on the first day of life. Massive right ventricular and main pulmonary artery dilatation are evident. RV, Right ventricle: MPA, main pulmonary artery: RPA, right pulmonary artery; LP.4, lef’t pulmonary artery.
3.
4.
5.
6.
7.
8.
9.
10. REFERENCES
Congcanital valvar regurgitation. In: Keith JD, Rowe RD, Vlad P, eds. Heart disease in infancy and childhood. .Ird ed. New York: MacMillan Publishing Co., 1978341. 2. Emmanouilides GC. Doroshow RW. Congenital absence ofthe
1.
251
Freedom RM.
11.
pulmonary valve. In: Adams FH, Emmanouilides (X, Riemenschneider TA. eds. Moss’ Heart disease in inf’ants, children and adolescents. 4th ed. Baltimore: Williams R- Wilkins. 1989:X01. Stanger I’. Lucas RV dr, Edwards .J. Anatomic features TRUSing respiratory distress in acyanotic congenital cardiac disease: special reference to bronchial obstruction. Pediatrics 1969;43:760-9. Smith RD, DuShane ,JW, Edwards ,JE. Congenital insufticiency of the pulmonary valve. including a case of fetal cardiac failure. (:irculation 1959:X):554-60. ItoT, Engle MA, Holswade GR. Congenital insuficiency ofthe pulmonic valve: a rare cause of neonatal heart failure. F’cdiatrics 1961;9X:712-8. Thanopoulos BD, Fisher EA, Hastreiter AR. Large ductus arteriosus and intact ventricular septum associated with congenital absence of the pulmonary valve. Br Heart J 19%: X:60”-4. Kron IL, Johnson AM, Carpenter MA. Gutgesell HP -Jr, Ovethold ED, Rheuban KS. Treatment of absent pulmonary valve syndrome with homograft,. Ann Thorac Surg 1988;46:57981. Alpert BS, Moore HV. “Absent” pulmonary valve with atria1 septal defect and patent ductus arteriosus. Pediatr Cardiol 1985;6:107-19. Sethia B. .Jamieson MPG, Houston AH. “Absent” pulmonar! valve with ASD and PDA (Letter). Pediatr C’ardiol 1986;7:1 II)20. Lau K, Cheung HH, Mok C. Congenital absence of the pulmonary valve. intact ventricular septum and patent duct us arteriosus: management in a newborn infant. AM HEAIU. .J 1990:120:711-14. Mair DD. Effect of markedly elevated hematcxrit level on blood viscosity and assessment ot pulmonary x-ascular renistame. .J Thorac Cardinvasc Surp 1979;77:682-4.
Brief Communications
Transesophageal echocardiography was extremely useful in following this patient postoperatively as well. On the seventh postoperative day transesophageal echocardiography revealed recurrence of vegetations on both mitral leaflets with abscess formation on the anterior mitral leaflet. Daniel et al.‘O found that transesophageal echocardiography was much more sensitive than transthoracic echocardiography in detecting abscess consequent upon endocarditis. After the detection of these abscesses and significant mitral regurgitation, the patient was taken for mitral valve replacement. The intraoperative transesophageal echocardiogram revealed that some of the multiple abscess spaces had ruptured to form a sinus tract through the anterior mitral leaflet. Recognition of pacemaker lead infection can be difficult. Transthoracic echocardiography is helpful, but image resolution may be inadequate in patients with small acoustic windows. While there are no large studies comparing the sensitivity of transthoracic echocardiography to transesophageal echocardiography in detecting pacemaker lead vegetations, this example, as well as the case reports by Van Camp et al.‘l and Zehender et a1.12 illustrates the detection of pacemaker lead vegetations by transesophageal echocardiography that were not visualized with transthoracic echocardiography. While transesophageal echocardiography offers significant advantages over transthoracic echocardiography in selected cases, it still has limitations in sensitivity, as in this case in which the vegetations on the tricuspid valve chordae and the right ventricular portion of the pacemaker lead were not visualized by transesophageal echocardiography but were subsequently found at surgery. The sensitivity of transesophageal echocardiography appears to be somewhat dependent on the number of views and planes in which the various structures are visualized. Transthoracic echocardiography will remain the standard of care when evaluating valvular competence and left ventricular function in patients with endocarditis having satisfactory acoustic windows. However, transesophageal echocardiography, because of its superior resolution and hence higher sensitivity in the detection of vegetations, should be considered as a valuable adjunct, especially in patients with poor acoustic windows or when complications are suspected. Serial examinations are also useful when surgery is planned or clinical deterioration occurs. REFERENCES
1. Yoshida K, Yoshikawa J, Yamaura Y, Hozumi T, Akasaka T, Fukaya T. Assessment of mitral regurgitation by biplane transesophageal color Doppler flow mapping. Circulation 1990;82:1121-6. 2. Lerner PI, Weinstein L. Infective endocarditis in the antibiotic era. N Engl J Med 1966;274:199-206. 3. Jung JY, Saab SB, Almond CH. The case for early surgical treatment of left-sided primary infective endocarditis. d Thorat Cardiovasc Surg 1975;70:509-18. 4. Jaffe WM, Morgan DE, Pearlman AS, Otto CM. Infective endocarditis, 1983-1988: echocardiographic findings and factors influencing morbidity and mortality. J Am Co11 Cardiol 1990;15:1227-33. 5. Martin RP. The diagnostic and prognostic role of cardiovas-
July 1992 Hean Journal
American
6.
7.
8.
9.
cular ultrasound in endocarditis: bigger is not hetter. .J .4m Co11 Cardiol 1990;15:1234-7. Stewart JA, Silimperi D, Harris P, Wise NK, Fraker TD, Kisslo JA. Echocardiographic documentation of vegetative lesions in infective endocarditis: clinical implications. Circulation 1980;61:375-80. Mugge A, Daniel WG, Frank G, Lichtlen PR. Echocardiography of infective endocarditis: reassessment of prognostic implications of vegetation size determined by the transthoracic and the transesophageal approach. .J Am Co11 Cardiol 1989;14:631-8. Erbel R, Rohmann S, Drexler M, Mohr-Kahaly S, Gerharz CD, Iversen S, Oelert H, Mever +J. Improved diagnostic value of echocardiography in patients with infective endocarditis by transesophageal approach. A prospective study. Eur Heart .J 1988;8:43-53. Taams MA, Gussenhoven EJ, Bos E, de daegere P, Roelandt .JRTC. Sutherland GR. Born N. Enhanced moroholonical diagnosis in infective endocarditis by transesophageal echocardiography. Br Heart J 1990;63:109-13. Daniel WG, Mugge A, Martin RP, Lindert 0, Hausmann I), Nonnast-Daniel B, Laas J, Lichtlen PR. Improvement in the diagnosis of abscesses associated with endocarditis by transesophageal echocardiography. N Engl J Med 1991:324:795-800. Van Camp G, Vandenbossche JL. Recognition of pacemaker lead infection by transesophageal echocardiography. Br Heart J 1990;65:229-30. Zehender M, Buchner C, Geibel A, Kasper W, Meinertz T, Just H. Diagnosis of hidden pacemaker lead sepsis by transesophageal echocardiography and a new technique for lead extraction. AM HEART J 1989;118:1050-3. I
10.
11.
12.
”
Congenital isolated pulmonary valve incompetence: Neonatal presentation and early natural history William Berman Jr., MD, Raymond R. Fripp, MD, Stuart A. Rowe, MD, and Steven M. Yabek, MD. Albuquerque, N.M.
Congenital dysplasia of the pulmonary valve leaflets, unassociated with a ventricular septal defect, is a rare congenital anomaly, having been described during life in about two dozen patients, only four of whom were neonates at the time of diagnosis. i, 2 We summarize our clinical and hemodynamic observations on two extensively studied neonates to define more clearly the presentation, management, and early natural history of this lesion. Case No. 1. I.G. weighed 3.9 kg. at birth, was cyanotic in room air, had an increase in respiratory effort, an active precordium, and a grade 416 to-and-fro systolic and diastolic murmur. Chest x-ray films showed marked cardiomegaly, and the electrocardiogram (ECG) showed severe right
From The University Pediatrics. Reprint requests: William of Pediatrics, UNMH-4N. 4f4137234
of New
Mexico
School
Berman Jr.. MD, Albuquerque,
of Medicine, UNM School NM 87131.
Department of Medicine,
of Dept.
Volume Number
124 1
Brief Communications
249
Fig. 1. A, Two-dimensional, short-axis echocardiographic view of right ventricular outflow tract, the pulmonary valve anulus, and the distal main pulmonary artery segment. B, Two-dimensional apical image demonstrating the relative sizes of the right ventricle and left ventricle. C, Two-dimensional short-axis image illustrating the size discrepancy between the main pulmonary and right branch pulmonary artery. Ao, Aortic root; Lt. left; LV, left ventricle; MPA, main pulmonary artery; MV, mitral valve; PAn, pulmonary artery; Rt, right; RV, right ventricle; RVOT, right ventricular outflow valve anulus; RPA, right pulmonary tract; TV, tricuspid valve; VS, ventricular septum. Table
I. Cardiac
catheterization Hb
Age
Hb, Hemoglobin; ovale.
R V pressure
fgmldli
Case No. 1 16 hr 1 Yr 2.8 yr 5.1 yr Case No. 2 1 day RV, right
data PA pressure
Systemic
(mmHd
(mmHd
pressure
(mmHg)
18 13 13 12
80/14 3615 3216 4416
63/30 no/10 22/10 24112
LV Ao Ao Ao
16
4814
35/10
LV = 5515
ventricle;
PA, pulmonary
artery;
Ao, aortic;
LV,
ventricular hypertrophy (RVH). An echocardiogram confirmed the presence of RVH, showed right ventricular cavity dilatation, a large main pulmonary artery, and abnormal appearing pulmonary valve leaflets with a minimally narrowed pulmonary valve anulus. No ventricular septal defects (VSD) was seen. Cardiac catheterization showed a restrictive patent ductus arteriosus (PDA), a small systolic pressure gradient across the pulmonary anulus, and mod-
= = = =
left ventricular;
Associated
72115 85160 105/60 112/81
PDA,
patent
lesions
Small None None None
PDA,
It to rt f.o. shunt
Small
PDA,
bidirectional
ductus
arteriosus;
It, left;
atria1 rt, right;
shunt
f.o., foramen
erately severe pulmonary valve incompetence (Table I). A loud systolic ejection click developed, and at 1 year of age repeat cardiac catheterization showed closure of the ductus arteriosus, a 16 mm Hg systolic pressure gradient across the pulmonic anulus, and mild pulmonary valve incompetence (Table I). The patient was recatheterized at age 3 years because of persistent cardiomegaly and increasing main pulmonary artery dilatation. The hemodynamics
250
Brief Communications
American
Heart
July 1992 Journal
Fig. 2. Color and continuous wave Doppler depictions of right ventricular outflow tract hemodynamics. A, View shows systolic turbulence beyond the level of the pulmonary valve anulus during systole. B, There is a broad diastolic incompetent jet through the valve anulus into the right ventricular outflow tract. C, A continuous wave Doppler tracing from the main pulmonary artery shows modest velocities of outflow turbulence (beneath the baseline) and pulmonary valve incompetence (above the baseline). m/s, meters/second.
were similar to those 2 years before (Table I). At 5 years of age, echocardiographic evidence of increasing pulmonary valve incompetence and right ventricular dilatation prompted the fourth cardiac catheterization. The systolic pressure gradient across the right ventricular outflow tract was slightly higher and the pulmonary valve incompetence was graded as moderate, but the tricuspid valve was competent and clearance of contrast from the right ventricle after angiography was brisk (Table I). The patient was last seen at 8% years of age and was asymptomatic. Case No. 2. A.G. weighed 3.1 kg at birth, was cyanotic but in little respiratory distress, and had an active precordium with systolic and diastolic thrills and a grade 4/6 to-and-fro systolic and diastolic murmur. A chest x-ray film showed massive main pulmonary artery segment dilatation; an ECG showed right ventricular hypertrophy. The echocardiogram showed massive right ventricular dilatation with dysplastic pulmonary valve leaflets and a 26 mm diameter
distal main pulmonary artery, but a 9 mm diameter pulmonary anulus (Fig. 1) and severe pulmonary valve incompetence (Fig. 2). The ventricular septum was intact. Cardiac catheterization showed massive right ventricular enlargement and main pulmonary artery dilatation (Fig. 3), a tortuous patent ductus arteriosus, trivial pulmonary annular stenosis, and severe pulmonary valve incompetence (Table I). Oxygen was administered to promote the physiologic fall in pulmonary vascular resistance. The patient showed no respiratory or cardiac distress over the first 3 months of life. Although cardiac physical findings currently are unchanged, the patient is asymptomatic and has nearly doubled his birth weight in 34’2 months. The term “absent pulmonary valve” in fact refers to a condition in which the pulmonary valve leaflets are rudimentary or dysplastic, the valve anulus is narrowed, and the hemodynamic consequence is mild right ventricular outflow tract obstruction and some degree of pulmonary
Volume
124
Number
1
valve incompetence. Some reports of congenital pulmonary valve absence refer to the syndrome in which pulmonary valve dysplasia coexists with a ventricular septal defect. This condition is referred to often as “tetralogy of Fallot with absent pulmonarv valve” and frequently is associated with neonatal respiral ory distress caused by airway compression hy aneurysmally dilated branch pulmonary art,erie$.” Little is known of the natural history of infants with isolated, congenital pulmonary valve “absence.” We can find only seven case reports of infants with absent pulmonar\’ valve and intact vent.ricular septum. The course and management of these infants have varied widely. Four died shorrly after birth (two of them during surgery), two underwent prosthetic valve insertion, and one had a large PDA ligated.a-“’ The two neonates we describe showed cyanosis but minimal respiratory distress and were managed conservatively because pulmonary valve incompetence (not stenosis) was the dominant lesion and the branch pulmonary arteries were not large enough to cause compression of the airways. In the presence of severe valvar regurgitalion, any attempt 1o reduce the systolic pressure gradient across the right ventricular outflow tract seemed unwise; because insertion of a prosthetic device is problematic in a neonate, and because airway obstruction was not severe, we treated the patients expect,antly. Severe respiratory distress did not develop, cyanosis and dyspnea resolved with time. and both patients are doing well without surgical intervention. At least three developmental processes are likely to promote hemodynamic improvemen: of this lesion with time. As the ductus arteriosus narrows and closes postnatally, the fetal aortopulmonary connection is eliminated. Consequently, both pulmonary flow and pressure will fall, reducing two of the factors promoting pulmonary valve incompetence. Second, with time and ventilation, pulmonary arteriolar resistance falls as a consequence of both remadelling and growth of the alveolar/ arteriolar units. This in turn permits pulmonary artery diastolic pressure to fall, reducing the driving pressure for pulmonary valve incompetence. Third, an independent and close relationship exists between hematocrit and calculated pulmonary vascular resistance.l’ With the postnatal, physiologic fall in hematocrit, a progressive reduction in pulmonary arteriolar resistance and pressure would be expected. The long-term outlook for these patients must be guarded. Intercurrent conditions that elevate pulmonary artery pressure can inr*rease t,he degree of pulmonary valve incompetence and precipitate right heart failure. Also, pulmonary valve incompetence may increase with time and cause tricuspid valve incompetence with eventual right heart failure. However, because physiologic, age-related factt:lrs promote improvement in infants with isolated, congenital “absence” of the pulmonary valve, we recommend conservative management unless respiratory distress makes surgical intervention unavoidable.
Brief Communications
Fig. 3. Still frame from a right ventricular angiogram performed in case No. 2 on the first day of life. Massive right ventricular and main pulmonary artery dilatation are evident. RV, Right ventricle: MPA, main pulmonary artery: RPA, right pulmonary artery; LP.4, lef’t pulmonary artery.
3.
4.
5.
6.
7.
8.
9.
10. REFERENCES
Congcanital valvar regurgitation. In: Keith JD, Rowe RD, Vlad P, eds. Heart disease in infancy and childhood. .Ird ed. New York: MacMillan Publishing Co., 1978341. 2. Emmanouilides GC. Doroshow RW. Congenital absence ofthe
1.
251
Freedom RM.
11.
pulmonary valve. In: Adams FH, Emmanouilides (X, Riemenschneider TA. eds. Moss’ Heart disease in inf’ants, children and adolescents. 4th ed. Baltimore: Williams R- Wilkins. 1989:X01. Stanger I’. Lucas RV dr, Edwards .J. Anatomic features TRUSing respiratory distress in acyanotic congenital cardiac disease: special reference to bronchial obstruction. Pediatrics 1969;43:760-9. Smith RD, DuShane ,JW, Edwards ,JE. Congenital insufticiency of the pulmonary valve. including a case of fetal cardiac failure. (:irculation 1959:X):554-60. ItoT, Engle MA, Holswade GR. Congenital insuficiency ofthe pulmonic valve: a rare cause of neonatal heart failure. F’cdiatrics 1961;9X:712-8. Thanopoulos BD, Fisher EA, Hastreiter AR. Large ductus arteriosus and intact ventricular septum associated with congenital absence of the pulmonary valve. Br Heart J 19%: X:60”-4. Kron IL, Johnson AM, Carpenter MA. Gutgesell HP -Jr, Ovethold ED, Rheuban KS. Treatment of absent pulmonary valve syndrome with homograft,. Ann Thorac Surg 1988;46:57981. Alpert BS, Moore HV. “Absent” pulmonary valve with atria1 septal defect and patent ductus arteriosus. Pediatr Cardiol 1985;6:107-19. Sethia B. .Jamieson MPG, Houston AH. “Absent” pulmonar! valve with ASD and PDA (Letter). Pediatr C’ardiol 1986;7:1 II)20. Lau K, Cheung HH, Mok C. Congenital absence of the pulmonary valve. intact ventricular septum and patent duct us arteriosus: management in a newborn infant. AM HEAIU. .J 1990:120:711-14. Mair DD. Effect of markedly elevated hematcxrit level on blood viscosity and assessment ot pulmonary x-ascular renistame. .J Thorac Cardinvasc Surp 1979;77:682-4.
