December 1978
The Journal
alP ED IA TR I CS
931
Echocardiographic changes in children with pulmonary hypertension secondary to upper airway obstruction When right ventricular ejection time and RV pre-ejection period are measured from the pulmonary valve echocardiogram, a RPEP I R VET ratio greater than 0.35 has been associated with increasedpulmonary vascular resistance. Four children with alveolar hypoventilation secondary to enlarged tonsils and adenoids, or Pierre Robin syndrome with microlarynx, were studied. Onset of upper airway obstruction ranged from birth to 2V2 years of age. The patients had a decreased Pao,
n9 10 60 mm Hg). elevated
Poco, (50 to 60 mm fIg] during sleep, right atrial and right ventricular hypertrophy by electrocardiogram, and cardiomegaly by roentgenogram. Pulmonary artery pressures during cardiac catheterization ranged from 75140 (m = 65] to 100/50 (m = 70) and RPEPIRVET was greater than 0.5 in each child (normal 0.24 ± 0.06). One pat/em, who \Vas not catheterized, had an RPEPIRVET
of 0.37. Oxygen administration or intubation during cardiac catheteriz ation reduced PAP 10 40110 (m = 30) and 50110 (m = 30). respectively, in t\Vo patients. and RP EP I R VET decreased simultaneously 10 less than 0.3. Three children underwent tonsillectomy and! or adenoidectomy and one child had
tracheostomy. RP EP! R VET decreased postoperatively 10 less than 0.31 in all patients. Sleep arterial blood gas values, ECG. and chest roentgenogram also reverted to normal.
Eliezer Nussbaum, M.D.,* Stephen S. Hirschfeld, M.D., Robert E. Wood, M.D., Ph.D., Thomas F. Boat, M.D., and Carl F. Doershuk, M.D., Cleveland, Ohio
secondary to hypoventilation due to enlarged tonsils and adenoids has become an established syndrome in the past decade. I. 2 The relationship between upper airway obstruction and increased pulmonary vascular resistance has been demonstrated noninvasively by physical examination, right atrial and right ventricular hypertrophy observed on serial electrocardiograms, and invasively by cardiac catheterization and arterial blood gases. Echocardiographic changes
PULMONARY HYPERTENSION
From Case Western Reserve University, School of Medicine, Department of Pediatrics, Rainbow Babies and Childrens Hospital. Supported in part by grants from United States Public Health Services No. HL 13885, HL 06009, and AM 08305, from the Cystic Fibrosis Foundation and United Torch Services. "Reprint address: Rainbow Babies and Childrens Hospital, 2101 Adelbert Rd.• Cleveland. OH 44106.
0022-3476178/120931 +06$00.60/0.q) 1978 The C. V. Mosby Co.
in right ventricular systolic time intervals have been useful in detecting the presence of pulmonary hypertension. When RV ejection time and RV pre-ejection period are Abbreviations used right ventricular RV: RPEP: right pre-ejection period RVET: right ventricular ejection time RVW: anterior right ventricular wall thickness LVD: left ventricular dimension RVD: right ventricular dimension ECO: electrocardiogram PAP: pulmonary artery pressure Pao.,: partial pressure of oxygen in arterial blood Pac~": partial pressure of carbon dioxide in arterial blood measured from a pulmonary valve echogram, a RPEP / RVET ratio greater than 0.35 (normal 0.24 ± 0.06) has been associated with increased pulmonary vascular resistance." Increased right ventricular wall thickness and
Vol. 93. No.6 pp. 931-936
932
The Journal of Pediatrics December 1978
Nussbaum et al.
111"1"11"1"'"11"1""11"11"111"111111111111111111"1"111"I111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111
EXP
INSP
rat io=O.65
ratio=O.40
.":-Fig. 1. Measurement of right ventricular systolic time intervals as demonstrated from the posterior pulmonary valve cusp echogram. The right pre-ejection period (RPEP) was measured from the onset of the QRS to the rapid descent of the pulmonary cusp. The right ventricular ejection time (R VET) was measured from pulmonary cusp opening to closure, the point at which the leaflet reapproaches its closed, thickened base line. There was marked phasic variation of the time intervals with respiration. dimension have also been associated with severe chronic obstructive pulmonary disease:" 5 This report evaluates the severity of pulmonary hypertension in upper airway obstruction employing echocardiography, which was found to be a simple, valuable modality in the care of children with severe upper airway obstruction. CASE REPORTS Patient 1. Patient S. J., a male with the Down syndrome, was seen for the first time at 71h months of age because of stridor and croup. Physical examination revealed an infant with noisy breathing and large tongue. Cyanosis was demonstrated with crying, and a marked right ventricular impulse and narrowly split S, with increased pulmonic component were noted. Nasolaryngoscopy revealed extreme anatomic narrowing at the midnasopharyngeal level. At 19 months of age adenoidectomy was performed with improvement in ventilation. Patient 2. Patient L. D., a 4~-year-old boy with the Down syndrome, presented at 21h years of age with chronic purulent rhinitis and sleep disturbance. Physical examination demonstrated enlarged tonsils and adenoids. Right ventricular impulse was increased; S, was single and accentuated. Evidence for failure of the right side of the heart was present (hepatomegaly and elevated jugular venous pressure). He underwent cardiac catheterization followed by adenoidectomy. Evidence of heart failure receded. One year later purulent rhinitis and nasopharyngeal obstruction recurred. The child underwent tonsillectomyand
adenoidectomy, followed by significant clinical improvement. Patient 3. Patient D. W. was born prematurely with a diagnosis of Pierre Robin syndrome and in the nursery developed respiratory distress, but did not need assisted ventilation. At 6, 10, 12, and 16 months of age she was hospitalized because of respiratory distress, cyanosis, noisy breathing, and congestive heart failure. Physical examination at that time revealed a posteriorly displaced large tongue, micrognathia, and cardiovascular findings consistent with cor pulmonale and failure of the right side of the heart. Nasolaryngoscopy with a small fiberoptic flexible bronchoscope showed a microlarynx. She was hospitalized with the same symptoms at 22 months of age. Following intubation and tracheostomy, dramatic clinical improvement was noted. Patient 4. Patient O. S. presented at 15 months of age with a history of chronic cough, daytime mouth breathing, and snoring at night. His physical examination revealed large tonsils and adenoids. Following tonsillectomy and adenoidectomy at 2 years of age. significant clinical improvement was noted.
METHODS Measurement of right ventricular and left ventricular systolic time intervals was accomplished from simultaneous, rapid speed (100 mru/second) recording of the electrocardiogram, pulmonary valve, and aortic echograms." In children with upper airway obstruction, there is often marked variation in RPEP IRVET from inspiration to expiration and the range ofRPEP/RVET, rather than a single value, should be reported (Figs. 1 and 2).
Volume 93 N umber 6
Echocardiographic changes with pulmonary hypertension
Fig. 2. Simultaneous tracings of the ECG, pulmonary arterial pressure, and pulmonary valve echo gram are recorded. The left panel was recorded during inspiration. The pulmonary arterial pressure was 60/25 with a RPEP IR VET of 0.33. The panel to the right was recorded in expiration ; the pressure of 80/60 corresponded with a RPEP/RvET of 0.58. Abbreviations used: EXP = Expiration ; INSF = inspiration; RPEP = right pre-ejection period; RVET = right ventricular ejection time.
Table I. Cardiac catheterization and arterial blood gas values pre- and postoperation Pulmonary artery pressure 01 catheterization ( Patient
2
3
Initial
Arterial blood gases Preoperative
)
Afte r 0 , administration
Aw ak e
75140 (m = 65)
pH Pao,
50110 (m = 30)
Pace,
90/40 (m = 60)
pH PaD, Paeo, pH Pao, Paeo , pH Pao, Paco, pH Pao, Paco ,
100/ 50 (m = 70)
40110 (m = 30) 4
Pao, = Partlal pressure of oxygen in arterial blood (mm Hgj: Paco, 'Before first operation. tBefore second operation (after first operation).
7.37' 57 35 7.32t 45
35 7 .33 60 40 7.32 43 56
I
Asleep
Postoperative
7.25 40 60
7.27 57
7.34t 39 54 7.40 55 50
= partial pressure of carbon dioxide in arterialb lood (mm Hg),
53
7.39 62
35 7.43 61 33-41 7.40
78 40
933
934
Nussbaum et al.
The Journal of Pediat rics December 1978
Table II. Echocardiographic measurements before and after operation After 0, administration
ECG Patien t
Chest w al l RV W
j
-RV 2
IVS
"- LV 3
4
Fig. 3-.The ultrasonic beam traverses a position through the body of the right ventricle (RV) and left ventricle (LV). All measurements were made at the onset of the QRS complex. Right ventricular wall (R VW) was measured from the undersurface of the chest wall to RV endocardium. RV dimension was measured from the RV endocardium to the right surface of the interventricular septum. There is marked thickening of the RVW and interventricular septum (IVS).
Measurements at end diastole (Q wave of ECG) of the right ventricular internal dimension and anterior wall thickness were made. The left ventricular dimension in diastole was likewise measured . Our patients were compared to previously described normal children': (Fig. 3). Pulmonary artery and aortic pressures were measured during cardiac catheterization. Sedation was achieved by using hydroxyzine hydrochloride (1 rug/kg) and meperidine hydrochloride (1 mg/kg). Systolic, diastolic, and mean PAP was measured using a No .5 Swan-Ganz flow directed catheter connected to Bell and Howell pressure transducers. Aortic pressures were measured in a: similar fashion with a Cook pigtail catheter. Echograms were recorded 18 hours prior to catheterization in Patients I and 3 and during catheterization in Patient 2. RESULTS Data concerning cardiac catheterization and arterial blood gas findings, and the serial echocardiograms are presented in Tables 1 and II, respectively. Significant
Pre-operative
RPEPI 0.50 RVET (N = 0.24 ± 0.06) RVW 0.6 (N = OJ) RVD 2.7 (N = 1.\ ± 0.2) LVD 2.1 eN = 2.8 ± 0.3) RPEPI 0.5 RVET eN = 0.14 ± 0.06) R VW 0.7 eN = 004) RVD 3.0(N = 1.1 ± D.2) LVD 2.\ (N = 3.3 ± D.3) RPEPI 0.66 RVET (N = 0.24 ± 0.06) RVW 0.6 (N = OJ) RVD 1.0 (N = 1.I ± 0.2) LVD 2.0 (N = 2.8 ± 0.3) RPEPI 0.37 RVET (N = 0.24 ± 0.06) RVW 0.3 eN = 0.3) RVD 1.2 (N = 1.1 :i: 0.2) LVD 2.5 eN = 2.8 ± 0.3)
or intubation
Postoperative
The Journal
alP ED IA TR I CS
931
Echocardiographic changes in children with pulmonary hypertension secondary to upper airway obstruction When right ventricular ejection time and RV pre-ejection period are measured from the pulmonary valve echocardiogram, a RPEP I R VET ratio greater than 0.35 has been associated with increasedpulmonary vascular resistance. Four children with alveolar hypoventilation secondary to enlarged tonsils and adenoids, or Pierre Robin syndrome with microlarynx, were studied. Onset of upper airway obstruction ranged from birth to 2V2 years of age. The patients had a decreased Pao,
n9 10 60 mm Hg). elevated
Poco, (50 to 60 mm fIg] during sleep, right atrial and right ventricular hypertrophy by electrocardiogram, and cardiomegaly by roentgenogram. Pulmonary artery pressures during cardiac catheterization ranged from 75140 (m = 65] to 100/50 (m = 70) and RPEPIRVET was greater than 0.5 in each child (normal 0.24 ± 0.06). One pat/em, who \Vas not catheterized, had an RPEPIRVET
of 0.37. Oxygen administration or intubation during cardiac catheteriz ation reduced PAP 10 40110 (m = 30) and 50110 (m = 30). respectively, in t\Vo patients. and RP EP I R VET decreased simultaneously 10 less than 0.3. Three children underwent tonsillectomy and! or adenoidectomy and one child had
tracheostomy. RP EP! R VET decreased postoperatively 10 less than 0.31 in all patients. Sleep arterial blood gas values, ECG. and chest roentgenogram also reverted to normal.
Eliezer Nussbaum, M.D.,* Stephen S. Hirschfeld, M.D., Robert E. Wood, M.D., Ph.D., Thomas F. Boat, M.D., and Carl F. Doershuk, M.D., Cleveland, Ohio
secondary to hypoventilation due to enlarged tonsils and adenoids has become an established syndrome in the past decade. I. 2 The relationship between upper airway obstruction and increased pulmonary vascular resistance has been demonstrated noninvasively by physical examination, right atrial and right ventricular hypertrophy observed on serial electrocardiograms, and invasively by cardiac catheterization and arterial blood gases. Echocardiographic changes
PULMONARY HYPERTENSION
From Case Western Reserve University, School of Medicine, Department of Pediatrics, Rainbow Babies and Childrens Hospital. Supported in part by grants from United States Public Health Services No. HL 13885, HL 06009, and AM 08305, from the Cystic Fibrosis Foundation and United Torch Services. "Reprint address: Rainbow Babies and Childrens Hospital, 2101 Adelbert Rd.• Cleveland. OH 44106.
0022-3476178/120931 +06$00.60/0.q) 1978 The C. V. Mosby Co.
in right ventricular systolic time intervals have been useful in detecting the presence of pulmonary hypertension. When RV ejection time and RV pre-ejection period are Abbreviations used right ventricular RV: RPEP: right pre-ejection period RVET: right ventricular ejection time RVW: anterior right ventricular wall thickness LVD: left ventricular dimension RVD: right ventricular dimension ECO: electrocardiogram PAP: pulmonary artery pressure Pao.,: partial pressure of oxygen in arterial blood Pac~": partial pressure of carbon dioxide in arterial blood measured from a pulmonary valve echogram, a RPEP / RVET ratio greater than 0.35 (normal 0.24 ± 0.06) has been associated with increased pulmonary vascular resistance." Increased right ventricular wall thickness and
Vol. 93. No.6 pp. 931-936
932
The Journal of Pediatrics December 1978
Nussbaum et al.
111"1"11"1"'"11"1""11"11"111"111111111111111111"1"111"I111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111111
EXP
INSP
rat io=O.65
ratio=O.40
.":-Fig. 1. Measurement of right ventricular systolic time intervals as demonstrated from the posterior pulmonary valve cusp echogram. The right pre-ejection period (RPEP) was measured from the onset of the QRS to the rapid descent of the pulmonary cusp. The right ventricular ejection time (R VET) was measured from pulmonary cusp opening to closure, the point at which the leaflet reapproaches its closed, thickened base line. There was marked phasic variation of the time intervals with respiration. dimension have also been associated with severe chronic obstructive pulmonary disease:" 5 This report evaluates the severity of pulmonary hypertension in upper airway obstruction employing echocardiography, which was found to be a simple, valuable modality in the care of children with severe upper airway obstruction. CASE REPORTS Patient 1. Patient S. J., a male with the Down syndrome, was seen for the first time at 71h months of age because of stridor and croup. Physical examination revealed an infant with noisy breathing and large tongue. Cyanosis was demonstrated with crying, and a marked right ventricular impulse and narrowly split S, with increased pulmonic component were noted. Nasolaryngoscopy revealed extreme anatomic narrowing at the midnasopharyngeal level. At 19 months of age adenoidectomy was performed with improvement in ventilation. Patient 2. Patient L. D., a 4~-year-old boy with the Down syndrome, presented at 21h years of age with chronic purulent rhinitis and sleep disturbance. Physical examination demonstrated enlarged tonsils and adenoids. Right ventricular impulse was increased; S, was single and accentuated. Evidence for failure of the right side of the heart was present (hepatomegaly and elevated jugular venous pressure). He underwent cardiac catheterization followed by adenoidectomy. Evidence of heart failure receded. One year later purulent rhinitis and nasopharyngeal obstruction recurred. The child underwent tonsillectomyand
adenoidectomy, followed by significant clinical improvement. Patient 3. Patient D. W. was born prematurely with a diagnosis of Pierre Robin syndrome and in the nursery developed respiratory distress, but did not need assisted ventilation. At 6, 10, 12, and 16 months of age she was hospitalized because of respiratory distress, cyanosis, noisy breathing, and congestive heart failure. Physical examination at that time revealed a posteriorly displaced large tongue, micrognathia, and cardiovascular findings consistent with cor pulmonale and failure of the right side of the heart. Nasolaryngoscopy with a small fiberoptic flexible bronchoscope showed a microlarynx. She was hospitalized with the same symptoms at 22 months of age. Following intubation and tracheostomy, dramatic clinical improvement was noted. Patient 4. Patient O. S. presented at 15 months of age with a history of chronic cough, daytime mouth breathing, and snoring at night. His physical examination revealed large tonsils and adenoids. Following tonsillectomy and adenoidectomy at 2 years of age. significant clinical improvement was noted.
METHODS Measurement of right ventricular and left ventricular systolic time intervals was accomplished from simultaneous, rapid speed (100 mru/second) recording of the electrocardiogram, pulmonary valve, and aortic echograms." In children with upper airway obstruction, there is often marked variation in RPEP IRVET from inspiration to expiration and the range ofRPEP/RVET, rather than a single value, should be reported (Figs. 1 and 2).
Volume 93 N umber 6
Echocardiographic changes with pulmonary hypertension
Fig. 2. Simultaneous tracings of the ECG, pulmonary arterial pressure, and pulmonary valve echo gram are recorded. The left panel was recorded during inspiration. The pulmonary arterial pressure was 60/25 with a RPEP IR VET of 0.33. The panel to the right was recorded in expiration ; the pressure of 80/60 corresponded with a RPEP/RvET of 0.58. Abbreviations used: EXP = Expiration ; INSF = inspiration; RPEP = right pre-ejection period; RVET = right ventricular ejection time.
Table I. Cardiac catheterization and arterial blood gas values pre- and postoperation Pulmonary artery pressure 01 catheterization ( Patient
2
3
Initial
Arterial blood gases Preoperative
)
Afte r 0 , administration
Aw ak e
75140 (m = 65)
pH Pao,
50110 (m = 30)
Pace,
90/40 (m = 60)
pH PaD, Paeo, pH Pao, Paeo , pH Pao, Paco, pH Pao, Paco ,
100/ 50 (m = 70)
40110 (m = 30) 4
Pao, = Partlal pressure of oxygen in arterial blood (mm Hgj: Paco, 'Before first operation. tBefore second operation (after first operation).
7.37' 57 35 7.32t 45
35 7 .33 60 40 7.32 43 56
I
Asleep
Postoperative
7.25 40 60
7.27 57
7.34t 39 54 7.40 55 50
= partial pressure of carbon dioxide in arterialb lood (mm Hg),
53
7.39 62
35 7.43 61 33-41 7.40
78 40
933
934
Nussbaum et al.
The Journal of Pediat rics December 1978
Table II. Echocardiographic measurements before and after operation After 0, administration
ECG Patien t
Chest w al l RV W
j
-RV 2
IVS
"- LV 3
4
Fig. 3-.The ultrasonic beam traverses a position through the body of the right ventricle (RV) and left ventricle (LV). All measurements were made at the onset of the QRS complex. Right ventricular wall (R VW) was measured from the undersurface of the chest wall to RV endocardium. RV dimension was measured from the RV endocardium to the right surface of the interventricular septum. There is marked thickening of the RVW and interventricular septum (IVS).
Measurements at end diastole (Q wave of ECG) of the right ventricular internal dimension and anterior wall thickness were made. The left ventricular dimension in diastole was likewise measured . Our patients were compared to previously described normal children': (Fig. 3). Pulmonary artery and aortic pressures were measured during cardiac catheterization. Sedation was achieved by using hydroxyzine hydrochloride (1 rug/kg) and meperidine hydrochloride (1 mg/kg). Systolic, diastolic, and mean PAP was measured using a No .5 Swan-Ganz flow directed catheter connected to Bell and Howell pressure transducers. Aortic pressures were measured in a: similar fashion with a Cook pigtail catheter. Echograms were recorded 18 hours prior to catheterization in Patients I and 3 and during catheterization in Patient 2. RESULTS Data concerning cardiac catheterization and arterial blood gas findings, and the serial echocardiograms are presented in Tables 1 and II, respectively. Significant
Pre-operative
RPEPI 0.50 RVET (N = 0.24 ± 0.06) RVW 0.6 (N = OJ) RVD 2.7 (N = 1.\ ± 0.2) LVD 2.1 eN = 2.8 ± 0.3) RPEPI 0.5 RVET eN = 0.14 ± 0.06) R VW 0.7 eN = 004) RVD 3.0(N = 1.1 ± D.2) LVD 2.\ (N = 3.3 ± D.3) RPEPI 0.66 RVET (N = 0.24 ± 0.06) RVW 0.6 (N = OJ) RVD 1.0 (N = 1.I ± 0.2) LVD 2.0 (N = 2.8 ± 0.3) RPEPI 0.37 RVET (N = 0.24 ± 0.06) RVW 0.3 eN = 0.3) RVD 1.2 (N = 1.1 :i: 0.2) LVD 2.5 eN = 2.8 ± 0.3)
or intubation
Postoperative
