The autopsy registry of congenital heart disease at the University of Padua.
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Autopsy↗
Biomedical subjects
S Y Ho
Publications and source records attributed to S Y Ho.
Absence of one atrioventricular connection associated with straddling atrioventricular valve: distinction of a solitary from a common valve and further considerations on the diagnosis of ventricular topology.
Hearts with absence of the right atrioventricular connection (tricuspid atresia) are rarely associated with straddling of the left atrioventricular valve. We describe two hearts in which the left atrium is connected mostly to a dominant left ventricle but, in part, to a rudimentary right ventricle. In these particular hearts, this arrangement caused major problems in determining ventricular topology. This feature, the so-called handedness of the ventricular mass, is determined in hearts with two complete ventricles by attempting to place the palm of one's hand on the right ventricular septal surface with the thumb in the inlet and the fingers pointing to the outlet. The problems in these two cases were produced because the rudimentary right ventricle had only part of an inlet component. The discerned pattern of topology was different in the two cases, being left hand in one and right hand in the other. These cases emphasize, first, the need to distinguish between ventricular topology and the atrioventricular connection and, second, between a solitary and a common atrioventricular valve when the valvar tension apparatus is straddling.
Heart Atria↗
The site of origin of nonconfluent pulmonary arteries from a common arterial trunk or from the ascending aorta: its morphological significance.
There are three possible embryological derivatives for nonconfluent pulmonary arteries which arise from a common arterial trunk or from the ascending aorta. We considered the feasibility of identifying these derivatives on the basis of the site of origin. We examined 15 specimens, in which both pulmonary arteries arose by separate orifices from a common arterial trunk (persistent truncus arteriosus), and 3 specimens, in which one pulmonary artery arose from the ascending aorta, the other being connected to the morphologically right ventricle (hemitruncus). Measurements were made for both the upper and lower margins of the orifices of the pulmonary arteries and then expressed as a percentage of the length of the ascending common trunk or aorta. The position of origin ranged from 39 to 100% for the upper margin and from 17 to 90% for the lower margin, without significant difference between left and right arteries. Since the range varied so widely, we contend that it is not possible to make a morphological identification of the pulmonary artery under consideration simply on the basis of the location of the site of origin.
Aorta↗
Further morphologic studies on tetralogy of Fallot, with particular emphasis on the prevalence and structure of the membranous flap.
We have investigated 158 hearts from patients with tetralogy Fallot so as to probe the precise anatomy of so-called membranous flaps and their relationship to the ventricular septal defect, the tricuspid valve, and the atrioventricular conduction axis. A membranous flap was detected in 61 hearts (38.6%) of the 158, all being found when the ventricular septal defect was perimembranous. Two types of flap were discovered. A remnant of the interventricular membranous septum (or a real flap) was seen in 32 and reduplication of the leaflets of the tricuspid valve (pseudoflap) was present in 32. The pseudoflaps either were connected to the anteroseptal commissure of the tricuspid valve or were attached to the medial papillary muscle complex. The medial papillary muscle was abnormally attached more frequently in those hearts with flaps (31%, 52%) than in the ones lacking a flap (5%) (p less than 0.01). An anomalous arrangement of the leaflet of the tricuspid valve was significantly more prevalent in those hearts with a pseudoflap (41%) than in those with a real flap (14%) and in those without a flap (9%). There was a tendency for real flaps to be smaller (p less than 0.05) and thinner than pseudoflaps. A few flaps were considered too tiny to warrant placement of sutures within their structure during surgical repair. During our investigation, we noted also the morphologic arrangement of the rest of the hearts.
Aortic Valve↗
Surgical implications of juxtaposition of the atrial appendages. A review of forty-nine autopsied hearts.
We studied 49 hearts with juxtaposition of the atrial appendages, concentrating on the associated malformations and features of operative importance. Left juxtaposition was found in 42 cases (86%) and right juxtaposition in seven cases (14%). In the group of hearts with right juxtaposition, a normal segmental arrangement was present in three cases, double inlet in two cases, and double-outlet right ventricle in the remaining two cases. Hearts with left juxtaposition had usual atrial arrangement with concordant atrioventricular connection, together with anomalous ventriculoarterial connection in 22 cases and univentricular atrioventricular connection in 19 cases. Only one specimen had a normal segmental arrangement. A partial form of left juxtaposition was present in 60% of the specimens. Hearts with left juxtaposition had a transverse orientation of the atrial septum, which was displaced posteriorly and inferiorly in the medial atrial wall, separated from the orifice of the juxtaposed appendage by a muscular band. A small right atrial chamber was noted in hearts with anomalous ventriculoarterial connection, creating the potential for anatomic difficulties during operation conducted within the atria. In two thirds of the cases with classic tricuspid atresia, the size of the orifice of the juxtaposed appendage was larger than the atrial septal defect, and an anastomosis of the appendage with the pulmonary trunk was morphologically feasible.
Adolescent↗
Coronary arterial and sinusal anatomy in hearts with a common arterial trunk.
Eighty-four specimens of common arterial trunk were studied with special reference to the arrangement of the leaflets in relation to the atrioventricular valves, the origin of the coronary arteries in relation to the arterial sinuses, and the epicardial course of the coronary arteries. Fourteen normal hearts were used for comparison. In the hearts with common arterial trunk, the location and level of the coronary artery orifices (as well as the relationship of the truncal root to the area of fibrous continuity with the mitral valve) are different from those in normal hearts. In none of the hearts with common arterial trunk (particularly the 53 hearts with three leaflets in the truncal valve) did the appearance of the truncal valve approximate that of a normal aortic valve. Among the 22 hearts with four leaflets, there was a high incidence of coronary artery orifices in opposite sinuses (17/22 or 77.3%) and a low incidence of coronary artery orifices in adjacent sinuses (2/22 or 9.1%). These results suggest that the formation of the truncal valve is independent of the formation of the coronary orifices. Its leaflets are not predestined to become part of either the aortic valve or the pulmonary valve.
Coronary Vessel Anomalies↗
Synergy of interleukin 1 (IL-1) with arachidonic acid and A23187 in stimulating PGE synthesis in human fibroblasts: IL-1 stimulates fibroblast cyclooxygenase.
The stimulation of prostaglandin E (PGE) synthesis by interleukin 1 (IL-1) has important physiologic effects in many target tissues. The mechanism(s) whereby IL-1 stimulates PGE synthesis is not well understood. Two alternative mechanisms have been postulated: hydrolysis of membrane phospholipid with release of arachidonic acid substrate and induction of cyclooxygenase activity. We examined these mechanisms in IL-1 stimulation of human dermal fibroblast PGE synthesis. IL-1 failed to induce release of previously incorporated radiolabeled arachidonic acid from fibroblasts under conditions where there was a 30-fold or greater stimulation of PGE synthesis. The calcium ionophore, A23187, gave much less stimulation of PGE synthesis while inducing 30-100% release of incorporated [14C]arachidonic acid. There was marked synergy between IL-1 and agents which increased availability of arachidonic acid. For example, the combination of IL-1 and A23187, used at concentrations where neither agent alone stimulated PGE synthesis, increased PGE synthesis from 3.1 to 22.5 ng/ml; similar synergy was seen between IL-1 and exogenous arachidonic acid. To examine a possible effect of IL-1 on cyclooxygenase synthesis, fibroblast cyclooxygenase was measured by radioimmunoassay. Following treatment with IL-1, fibroblasts demonstrated a two- to threefold increase in content of immunoreactive cyclooxygenase. These results suggest that IL-1 increases fibroblast PGE synthesis by a mechanism(s) other than making available increased membrane arachidonic acid, and that at least part of its effect may be mediated by induction of cyclooxygenase. Furthermore, the effect of IL-1 on fibroblast PGE synthesis is markedly potentiated by agents which increase available substrate.
Arachidonic Acid↗
A rare case of tetralogy of Fallot with unusual blood supply to the left lung.
We present a rare case of tetralogy of Fallot with absence of the leaflets of the pulmonary valve and anomalous blood supply of the left lung from the transverse aortic arch. Although embryologic considerations would suggest that the anomalous vessel is not the arterial duct, there was a good response to prostaglandin therapy.
Angiography↗
Cardiovascular cavities cast in silicone rubber as an adjunct to post-mortem examination of the heart.
We used silicone rubber as a material for making post-mortem casts of cavities in 21 human hearts, 16 of them congenitally malformed, 13 after surgical reconstruction (Fontan and Norwood procedures) and 4 after reconstruction had been performed posts mortem for study purposes. The organs were either fresh, or had been perfusion fixed with formalin prior to casting. When suitable silicone rubber and catalyst were used, we found that casts allowed a very clear view of chamber morphology, and, where surgery had been performed, of any vascular constriction or distortion. We made casts of the aortic sinus and coronary arteries in 3 adult hearts, demonstrating the location and extent of indentation due to atheromatous deposits. The resilience and non-adhesiveness of the silicone rubber allowed casts to be extracted without recourse to corrosion. The surrounding tissues, with the exception of trapped trabeculations and valve structures, were then available for conventional pathological examination. We propose that the relatively simple technique has important applications, not practicable using earlier casting materials, for the study of cardiovascular morphology, and for post-mortem evaluation of stenosed or surgically reconstructed vessels.
Adult↗
Persistent 5th aortic arch--a great pretender: three new covert cases.
Although persistence of the embryonic fifth aortic arch is considered a rare congenital malformation, analysis of the records of the cardiopathological collections of the Brompton and Killingbeck Hospitals reveals 6 examples in approximately 2000 specimens, an incidence amongst our material of 1 in 330. Since our review of the literature reveals only a further 13 described cases, we wonder if the condition may go unrecognized rather than being exceedingly rare. This possibility is further supported by the fact that 3 of our cases were incorrectly interpreted when initially described. In this report, we describe the details of 2 of these specimens and one other case that was not included in our earlier descriptions of this malformation. The first case had the persistent fifth arch as a conduit between the pulmonary and systemic circulations in the setting of aortic atresia with interruption of the aortic arch. The second case demonstrated a double lumen aortic arch, the fifth arch in this instance being an accessory systemic-to-systemic conduit. In the final case, the persistent arch was initially considered to represent an aorto-pulmonary window but review revealed an aortic to pulmonary conduit more in keeping with the presence of a fifth arch. We conclude that the powers of mimicry of this enigmatic structure may account for its apparent rarity.
Aorta, Thoracic↗
Endocarditis in an infant causing "tricuspid atresia".
A 3-month-old male infant died of tricuspid valve nonbacterial thrombotic endocarditis complicating disseminated intravascular coagulation. The vegetations were so extensive as to cause tricuspid atresia and led to congestive right ventricular failure terminating in death. The diagnosis was made only at post mortem.
Endocarditis↗
Essential fatty acid and lipid profiles in plasma and erythrocytes in patients with multiple sclerosis.
This study was conducted to investigate the possible differences in erythrocyte lipid composition, which might account for the previously reported increase in erythrocyte membrane zinc levels in patients with multiple sclerosis (MS). Compared with healthy control subjects, plasma lipids in patients with MS contained less sphingomyelin but more phosphatidylserine and the cholesterol-phospholipid ratio was 42% higher in the plasma from MS patients (p less than 0.01). In erythrocytes from MS patients, phosphatidylinositol was lower and erythrocyte cholesterol per milligram protein was significantly lower than concentrations in healthy control subjects (p less than 0.01). Among the long-chain fatty acids, the omega-3 fatty acids were lower in plasma from MS patients and linoleic acid was lower in erythrocyte ghosts from MS patients (p less than 0.01). We conclude that altered levels of cholesterol in plasma and erythrocytes from MS patients may contribute to increased erythrocyte-membrane Zn in MS patients. It cannot be stated with certainty whether the altered fatty acid profiles in MS patients were a function of the disease or of altered fatty acid intake.
Cholesterol↗
Anomalous subaortic position of the brachiocephalic (innominate) vein: a review of published reports and report of three new cases.
Anomalous courses of the left innominate vein have rarely been described in anatomical specimens. Investigative techniques such as angiography and echocardiography have brought to light more instances of this anomaly. Three more cases identified by anatomical study are described. Earlier cases were reviewed to assess the type of associated cardiac malformations. Clinically, the abnormality is regarded as benign. When it is recognised during investigation it should alert the clinician to the possibility of associated malformations. Features commonly seen in tetralogy of Fallot--right aortic arch, ventricular septal defect, and right ventricular outflow obstruction--were common in patients with anomalous subaortic innominate veins.
Brachiocephalic Veins↗
Morphological nature of the atrioventricular valves in hearts with double inlet left ventricle.
The rudimentary right ventricle in hearts with double inlet to a dominant left ventricle can exist either to the right or the left side of the ventricular mass. These variants have been interpreted to imply differences in ventricular topology. If correct, they also imply that differences should exist in the morphology of the atrioventricular valves, previously thought to be indistinguishable one from the other. To test this possibility, we examined 15 hearts with double inlet to a morphologically left ventricle and 10 normal hearts. The distinguishing features of normal valves were evaluated for their consistency, and the most reliable were employed for interpreting the valves in the abnormal hearts. As shown by previous echocardiographic studies, insertion of valvar tension apparatus to the interventricular septum was unique to the morphologically tricuspid valve. Ten of the abnormal hearts had one valve showing this feature, it being absent in the other valve. The attachment, consistently on the same side as the rudimentary right ventricle, could be of diagnostic value in determining ventricular topology. In our small study, nonetheless, this approach still leaves one third of the hearts in which topology cannot be ascertained with certainty and in which it is not possible to distinguish the morphological nature of the atrioventricular valves.
Echocardiography↗
[Comparative study on the reflex responses of carotid and aortic baroreceptors in the rabbit].
In 81 anesthetized rabbits, the baroreflex control of heart rate (HR), hind-limb vascular resistance (HVR) and renal sympathetic nerve activity (RSNA) was observed during arterial baroreceptor loading and unloading by intravenously injecting phenylephrine (PE) and nitroprusside (NP). The results were as follows: (1) An increase of arterial pressure with PE caused reduction in HR, HVR and RSNA, while a decrease of arterial pressure with NP evoked opposite responses. These reflex responses were reproducible. (2) By either carotid baroreceptor denervation (CBRX) or aortic baroreceptor denervation (ABRX), the reflex changes of HR induced by injecting PE and NP were impaired (P less than 0.01), while the reflex responses in HVP remained unchanged. Despite of the enhanced basal RSNA following ABRX or CBRX, the magnitude of reflex inhibition in RSNA during injecting NP was similar to that before denervation, whereas that of the reflex excitation in RSNA during injecting NP was reduced (P less than 0.05). (3) After complete sino-aortic denervation (SAD), the change of arterial pressure following PE or NP injection was enhanced, but the reflex changes in HR, HVR and RSNA were significantly diminished (P less than 0.001). (4) Vagotomy abolished the residual reflex changes observed after SAD. The results indicate that the aortic and carotid baroreceptors may regulate HR in a simple additive manner, while the aortic baroreceptor seems to be more important. Furthermore, both the aortic and carotid baroreceptors may play important roles for the reflex control of HVR and RSNA, and operate mutually by the way of inhibitory summation.
Animals↗
[Arterial baroreflex in the renal hypertensive rabbits].
In 14 anesthetized male rabbits subjected bilateral renal wrapping 12 weeks before, the baroreflex control of heart rate (HR), hindlimb vascular resistance (HVR) and renal sympathetic nerve activity (RSNA) was examined during arterial baroreceptor loading and unloading by injecting phenylephrine (PE) and nitroprusside (NP). The results were as follows: (1) The mean arterial pressure (131 +/- 9 mmHg) of the kidney-wrapped rabbits was significantly higher than that (95 +/- 10 mmHg) of the control rabbits (P less than 0.001). (2) With the buffer nerves intact, the baroreflex control of HR was obviously impaired in the hypertensive rabbits (P less than 0.01), whereas the reflex control of HVR and RSNA remained unchanged as compared with the normotensive rabbits (P greater than 0.05), indicating a dissociation of baroreflex control of HR from that of peripheral circulation. However, in response to local intraarterial injection of PE and NP, the changes in HVR showed no difference between the hypertensive and normotensive rabbits. (3) After aortic or carotid baroreceptor denervation, the reflex control of HR was further reduced in the hypertensive rabbits, and the reflex control of HVR and RSNA was also significantly reduced (P less than 0.01), suggesting an impairment of potential baroreflex regulation of the circulation. It is likely that the changes of baroreflex in hypertensive rabbits may be attributed to the abnormalities in both baroreceptor and central integrating mechanism.
Animals↗
[Hemodynamic effects of the Bezold-Jarisch reflex in rabbit].
The Bezold-Jarisch reflex induced by intracoronary injection of nicotine (ICIN) of 5 micrograms/kg was studied in 40 anesthetized rabbits. Heart rate (HR), arterial blood pressure (ABP), left ventricular pressure (IVP), left ventricular dp/dt (LV dp/dt) and cardiac output (CO) were monitored and total peripheral resistance (TPR) was calculated. HR, ABP, LVP and LV dp/dt were decreased following ICIN. These inhibitory responses induced by ICIN were enhanced by sino-aortic denervation but abolished by bilateral vagotomy. Decreases in CO and TPR were also observed in response to ICIN. With the heart paced artificially to prevent bradycardia, the decreases in CO, TPR and ABP were still significant. Bilateral stellate ganglion section plus atropinization which abolished the decreases in HR and CO, did not significantly change the hypotensive response to ICIN. These data suggest that the hypotension evoked by Bezold-Jarisch reflex in rabbit is largely due to the decreases in TPR and partially caused by the decrease in CO.
Animals↗
[Change of sympathetic efferent discharges in different regions during the Bezold-Jarisch reflex in rabbit].
In 46 anesthetized rabbits, the sympathetic efferent discharges in different vascular regions were recorded during the Bezold-Jarisch reflex induced by intracoronary injection of nicotine. The discharges of renal nerve, cardiac nerve, splenic nerve, ramus of stellate ganglion to cervical plexus and ramus of superior cervical ganglion to external carotid artery decreased significantly following injection of nicotine. The decrease in efferent discharge is more prominent in renal, cardiac and splenic nerves than in the other two nerves. These results demonstrated that the reduction in discharge of sympathetic efferent fibers of different regions and hence decrease in total peripheral resistance might play a significant role in initiating hypotension during the Bezold-Jarisch reflex.
Animals↗