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Aortic valve atresia: A study of 73 necropsy patients
ABSTRACTS
AORTIC VALVE ATRESIA: A STUDY OF 73 NECROPSY PATIENTS William C. Roberts, MD, FACC, National Heart & Lung Institute, Bethesda, Maryland, and Lowell W. Perry, MD, FACC, Roma S. Chandra, MD, Gene E. Myers, MD, Stephen R. Shapiro, MD, and Lewis P. Scott, III, MD, FACC, Children's Hospital National Medical Center, Washington, D.C. Aortic valve atresia (AVA) is the least tolerated cardiac anomaly, and, among patients (pts) with congenital heart disease, the most common cause of congestive cardiac failure and death during the first week of life. Of the 73 pts, the age at death ranged from 1 to 56 days (avg 7) and 80% died during the first week; 70% were boys. Of the 73 pts, 69 (95%) had hypoplastic left ventricles (LV) with intact ventricular septa (VS) (Type I): the mitral valves @IV) in 25 were atretic (Type IA) and in 44, hypoplastic (Type IB). In each of the remaining 4 pts, the LV was well developed and one or more defects were present in the VS (Type II): the MV in 1 was atretic (Type IA) and in 3, well-developed (Type IB). Thus, the major variable in pts with AVA is the status of the VS: when intact, the LV is hypoplastic; when one or more defects are present in the VS. the LV is well developed. Normal or near-normal development of the LV has not been described previously in AVA. When the LV is developed, operation is of potential benefit, but when hypoplastic, operation is futile.
EFFECT OF HEPARIN-INDUCED ELEVATION OF FREE FATTY ACIDS ON MYOCARDIAL OXYGEN CONSUMPTION IN MAN. William J. Rogers, MD; Richard 0. Russell, Jr., MD, FACC: Roger E. Moraski, MD; Huey G. McDaniel, MD; Charles E. Rackley, MD, FACC. Univ of Alabama Med Ctr, Birmingham, Alabama. Recent studies in experimental animals have suggested that the acute elevation of free fatty acids (FFA) following heparin-induced lipolysis might significantly augment myocardial oxygen consumption (MV02). If applicable to man, this observation would pose a relative contraindication to the use of heparin in patients (pts) with coronary artery disease (CAD). Accordingly, the acute metabolic consequences of post-heparin lipolysis were studied in 17 pts-9 pts following a 12 hr fast (Gp A), and 8 pts 3 hrs after a 100 gm fat meal (Gp B). Baseline measurements included heart rate, mean aortic pressure, coronary sinus blood flow (thermodilution technique), and arterial-coronary sinus FFA, oxygen and C02. A bolus of 5000 IJ heparin was given IV, and all measurements were repeated 30 min later. Of the 17 pts, 13 were later found to have CAD. RESULTS: Marked arterial FFA elevation occurred at 30 min post-heparin in both groups (Gp A: 364 f 53 to 924 + 131 mEq/L, pc.001; Gp B: 479 * 48 to 1842 r 168 mEq/L, pc.001). Myocardial FFA A-V differences also increased significantly (Gp A: 78 f 24 to 175 f 31 mEq/L, pc.10; Gp B: 76 ? 20 to 189 + 41 mEq/L, pc.10). MV02 was unchanged in both groups (Gp A: 18.11 f 0.83 to 17.59 ?: 0.76 ml/min, p NS; Gp 8: 20.48 f 2.90 to 19.80 ? 2.60 ml/min, p NS). No significant changes in either group occurred in heart rate mean aortic pressure, coronary sinus flow, or respiratory quotient. Metabolic findings in pts with normal coronary anatomy and CAD were similar. CONCLUSION: Despite marked acute plasma FFA elevation in man following a therapeutic bolus of IV heparin, there is no significant alteration in myocardial oxygen consumption compared to control values.
LEFT VENTRICULAR FUNCTION AFTER SUXICAL PEPAIR OF TETRALOGY OF FALLOT Albert S. Rocchini, MD; John F. Keane, MD; Michael D. Freed, MD; Aldo R. Castaneda, MD; Alexander S. Nadas, MD; Children's Hospital Medical Center Boston, Ma.
QUANTITATION OF HUMAN A-V NODAL CONCEALED CONDUCTION UTILIZING SlS2S3 STIMULATION Kenneth Rosen, MD, FACC; Delon Wu, MD; Pablo Denes, MD, FACC; C.R.C. Wyndham, MD; F. Amat-y-Leon, MD; Ramesh Dhingra , MD, FACC, University of Illinois, Chicago, Illinois.
Left Ventricular (LV) function was assessed by angiographic methods in 41 patients (pts) (ages4-261, l-12 yrs. following repair of Tetralogy of Fallot (S/P TOF). ?trenty of the 41 pts (Group A) had a good repair with a pulmonary to systemic flow ratio (Qp/Qs) < 1.5 and a right ventricular pressure (RVP) of<2/3 systemic levels. The other 21/41 pts (Group B), had a Qp/Qs >1.5 and/or RVP>2/3 systemic. Eight of the pts from Group B with an isolated residual ventricular septal defect (VSD) (Qp/Qs 2) were canpared to 8 pts with an isolated large VSD, matched for body surface area and Qp/Qs. The end-diastolic volumes (EDV) ejection fractions (EF), and LV masses LVM are as follows:
Concealed conduction of atria1 extra-stimuli (A21 blocked in the A-V node was studied in 8 patients (pts) utilizing a third stimulus (A3), coupled to A2 at decreasing coupling intervals. Three Al-A2 intervals were tested in each pt: long (just shorter than the A-V nodal effective refractory period) (mean 412 msec), intermediate (mean 348 msec) and short (equal to the atria1 functional refractory period) (mean 301 msec). A-V nodal refractory period (AVN-RP) after A2 was defined as the longest AZ-A3 not conducted to the His bundle. The following curves were constructed for each A2: Al-A3, Hl-H3 and AZ-A3, A3-H3. The former curve was also compared to control Al-A2, Hl-H2. Concealment of A2 was demonstrated at all tested Al-AZ. In 4 pts, AVN-P.P was longer at longest Al-AZ, suggesting deeper penetration of late A2. In 2 pts, AVN-RP was equal at all Al-A2 suggesting equal penetration of A2 despite difference in timing. In 2 pts, AVN-RP was longest at shortest Al-A2, suggesting deeper penetration of early A2. In all 8 pts, A2-A3, A3-H3 curves were similar at the three tested Al-A2. In conclusion, the three types of response suggest that the level of concealment varies and cannot be predicted from the timing of a blocked A2. Concealment of A2 is more apparent from analysis of AVN-RP than conduction times following A2.
EDV (cc/m21 N;?& EF LVM (g/m21 EDV (cc/m2) z (g,m2)
m
m
These findings indicate that pts with good repair of TCF (Group A) have normal LV function. Those pts S/P TOF with large residual VSD's have impaired LV function especially when compared with pts with uncanplicated VSD with a similar Qp/Qs.
January 1976
The American Journal of CARDIOLOGY
Volume 37
167
AORTIC VALVE ATRESIA: A STUDY OF 73 NECROPSY PATIENTS William C. Roberts, MD, FACC, National Heart & Lung Institute, Bethesda, Maryland, and Lowell W. Perry, MD, FACC, Roma S. Chandra, MD, Gene E. Myers, MD, Stephen R. Shapiro, MD, and Lewis P. Scott, III, MD, FACC, Children's Hospital National Medical Center, Washington, D.C. Aortic valve atresia (AVA) is the least tolerated cardiac anomaly, and, among patients (pts) with congenital heart disease, the most common cause of congestive cardiac failure and death during the first week of life. Of the 73 pts, the age at death ranged from 1 to 56 days (avg 7) and 80% died during the first week; 70% were boys. Of the 73 pts, 69 (95%) had hypoplastic left ventricles (LV) with intact ventricular septa (VS) (Type I): the mitral valves @IV) in 25 were atretic (Type IA) and in 44, hypoplastic (Type IB). In each of the remaining 4 pts, the LV was well developed and one or more defects were present in the VS (Type II): the MV in 1 was atretic (Type IA) and in 3, well-developed (Type IB). Thus, the major variable in pts with AVA is the status of the VS: when intact, the LV is hypoplastic; when one or more defects are present in the VS. the LV is well developed. Normal or near-normal development of the LV has not been described previously in AVA. When the LV is developed, operation is of potential benefit, but when hypoplastic, operation is futile.
EFFECT OF HEPARIN-INDUCED ELEVATION OF FREE FATTY ACIDS ON MYOCARDIAL OXYGEN CONSUMPTION IN MAN. William J. Rogers, MD; Richard 0. Russell, Jr., MD, FACC: Roger E. Moraski, MD; Huey G. McDaniel, MD; Charles E. Rackley, MD, FACC. Univ of Alabama Med Ctr, Birmingham, Alabama. Recent studies in experimental animals have suggested that the acute elevation of free fatty acids (FFA) following heparin-induced lipolysis might significantly augment myocardial oxygen consumption (MV02). If applicable to man, this observation would pose a relative contraindication to the use of heparin in patients (pts) with coronary artery disease (CAD). Accordingly, the acute metabolic consequences of post-heparin lipolysis were studied in 17 pts-9 pts following a 12 hr fast (Gp A), and 8 pts 3 hrs after a 100 gm fat meal (Gp B). Baseline measurements included heart rate, mean aortic pressure, coronary sinus blood flow (thermodilution technique), and arterial-coronary sinus FFA, oxygen and C02. A bolus of 5000 IJ heparin was given IV, and all measurements were repeated 30 min later. Of the 17 pts, 13 were later found to have CAD. RESULTS: Marked arterial FFA elevation occurred at 30 min post-heparin in both groups (Gp A: 364 f 53 to 924 + 131 mEq/L, pc.001; Gp B: 479 * 48 to 1842 r 168 mEq/L, pc.001). Myocardial FFA A-V differences also increased significantly (Gp A: 78 f 24 to 175 f 31 mEq/L, pc.10; Gp B: 76 ? 20 to 189 + 41 mEq/L, pc.10). MV02 was unchanged in both groups (Gp A: 18.11 f 0.83 to 17.59 ?: 0.76 ml/min, p NS; Gp 8: 20.48 f 2.90 to 19.80 ? 2.60 ml/min, p NS). No significant changes in either group occurred in heart rate mean aortic pressure, coronary sinus flow, or respiratory quotient. Metabolic findings in pts with normal coronary anatomy and CAD were similar. CONCLUSION: Despite marked acute plasma FFA elevation in man following a therapeutic bolus of IV heparin, there is no significant alteration in myocardial oxygen consumption compared to control values.
LEFT VENTRICULAR FUNCTION AFTER SUXICAL PEPAIR OF TETRALOGY OF FALLOT Albert S. Rocchini, MD; John F. Keane, MD; Michael D. Freed, MD; Aldo R. Castaneda, MD; Alexander S. Nadas, MD; Children's Hospital Medical Center Boston, Ma.
QUANTITATION OF HUMAN A-V NODAL CONCEALED CONDUCTION UTILIZING SlS2S3 STIMULATION Kenneth Rosen, MD, FACC; Delon Wu, MD; Pablo Denes, MD, FACC; C.R.C. Wyndham, MD; F. Amat-y-Leon, MD; Ramesh Dhingra , MD, FACC, University of Illinois, Chicago, Illinois.
Left Ventricular (LV) function was assessed by angiographic methods in 41 patients (pts) (ages4-261, l-12 yrs. following repair of Tetralogy of Fallot (S/P TOF). ?trenty of the 41 pts (Group A) had a good repair with a pulmonary to systemic flow ratio (Qp/Qs) < 1.5 and a right ventricular pressure (RVP) of<2/3 systemic levels. The other 21/41 pts (Group B), had a Qp/Qs >1.5 and/or RVP>2/3 systemic. Eight of the pts from Group B with an isolated residual ventricular septal defect (VSD) (Qp/Qs 2) were canpared to 8 pts with an isolated large VSD, matched for body surface area and Qp/Qs. The end-diastolic volumes (EDV) ejection fractions (EF), and LV masses LVM are as follows:
Concealed conduction of atria1 extra-stimuli (A21 blocked in the A-V node was studied in 8 patients (pts) utilizing a third stimulus (A3), coupled to A2 at decreasing coupling intervals. Three Al-A2 intervals were tested in each pt: long (just shorter than the A-V nodal effective refractory period) (mean 412 msec), intermediate (mean 348 msec) and short (equal to the atria1 functional refractory period) (mean 301 msec). A-V nodal refractory period (AVN-RP) after A2 was defined as the longest AZ-A3 not conducted to the His bundle. The following curves were constructed for each A2: Al-A3, Hl-H3 and AZ-A3, A3-H3. The former curve was also compared to control Al-A2, Hl-H2. Concealment of A2 was demonstrated at all tested Al-AZ. In 4 pts, AVN-P.P was longer at longest Al-AZ, suggesting deeper penetration of late A2. In 2 pts, AVN-RP was equal at all Al-A2 suggesting equal penetration of A2 despite difference in timing. In 2 pts, AVN-RP was longest at shortest Al-A2, suggesting deeper penetration of early A2. In all 8 pts, A2-A3, A3-H3 curves were similar at the three tested Al-A2. In conclusion, the three types of response suggest that the level of concealment varies and cannot be predicted from the timing of a blocked A2. Concealment of A2 is more apparent from analysis of AVN-RP than conduction times following A2.
EDV (cc/m21 N;?& EF LVM (g/m21 EDV (cc/m2) z (g,m2)
m
m
These findings indicate that pts with good repair of TCF (Group A) have normal LV function. Those pts S/P TOF with large residual VSD's have impaired LV function especially when compared with pts with uncanplicated VSD with a similar Qp/Qs.
January 1976
The American Journal of CARDIOLOGY
Volume 37
167