Standard 12 lead and 24 hour Holter electrocardiographic observation in a biracial group of perinatally asphyxiated newborns.

AIM: To compare the cardiovascular responses to perinatal stress between two ethnic groups of newborns. STUDY POPULATION: 23 Nigerians and 14 Dutch perinally asphyxiated newborns together with their respective control of 12 healthy Nigerian and 16 Dutch newborns. METHOD: The study subjects and their controls were evaluated with standard 12-lead and 24-hour Holter electrocardiographic recordings within 36 hours of postnatal life. RESULT: Rhythm, rate, P-wave, P-R interval and QRS duration were normal in study subjects and their respective controls on standard ECG. The prevalence of ST-segment depression in the mid-precordial lead V4 was significantly higher in the asphyxiated Nigerian infants (78%) than in the controls (42%); p < 0.05. Similarly, its prevalence in the Dutch infants (50%) was significanly different from the zero prevalence in the Dutch controls; p < 0.05. Long duration ECG recordings showed a 'fixed heart rate' phenomenon in the asphyxiated infants but not in their controls. CONCLUSION: ST-segment depression may imply myocardial ischaemia in asphyxiated Dutch newborns but such may not be inferred in asphydiated Nigerian newborns. 'Fixed heart rate' phenomenon or loss of sinus arrhythmia appears to be causally related to hypoxaemia which results from perinatal asphyxia.

Serum creatine kinase and creatine kinase-MB isoenzyme activities in perinatally asphyxiated newborns.

Serum creatine kinase (CK) and creatine kinase-MB isoenzyme (CK-MB) activities were studied prospectively in serial blood samples obtained from 23 perinatally asphyxiated negroid newborns and 12 healthy controls during the first 100 h of life. The asphyxiated infants had significantly elevated mean CK and absolute CK-MB but no fractional CK-MB activities. Peak mean CK and CK-MB values (U.l-1) were 789.17 (+/- 220), P less than 0.01 and 16.36 (+/- 3.0) P less than 0.001 respectively at the 6-8 post-partum period. The healthy controls, on the other hand, showed a steady decline in the activities of these enzymes from birth. The vaginally and operatively delivered asphyxiated infants showed significantly higher CK and CK-MB activities than their respective non-asphyxiated controls, but no increase in fractional CK-MB was recorded in any of the groups. The elevation in absolute and fractional CK-MB 42.0 U.l-1 (5.1%) in respect of the infants with TTI (transient tricuspid incompetence) was significant (P less than 0.05) when compared with the controls with features of TTN (transient tachypnoea of the newborn) in the 6-8 h post-partum period. One of the infants with TTI at autopsy had hypoxic myocardial injury. The specificity of CK-MB, as a marker of myocardial injury in asphyxiated negroid neonates, is plausible but remains uncertain. Until the lack of rise of CK and CK-MB in healthy negroid newborns is confirmed in a larger series with further studies on MM, BB and MB isoenzymes, caution is urged in the interpretation of elevated CK and CK-MB activities in asphyxiated negroid newborns.

Method for the computation of an accurate zero reference for ECG signals.

When the instantaneous magnitude of an electrical signal is to be determined, a reference has to be known against which the signal value should be measured. In electrocardiology, this reference is commonly derived from the isoelectrical period in the ECG. However, this period is not a clearly defined part of the ECG, and its use can be problematic. The signal level obtained from it is often not sufficiently unambiguous and in the case of a high heart rate this period is too short to be used or not present at all. In the paper a method is developed to compute a reference value, independent of the isoelectrical period and of heart rate. The method is derived from a method published before, which we developed for the separation of the surface His-Purkinje signal from the P-wave in the case of overlap. In the present application of the method, two successive heartbeats are separated in an analogous manner, revealing the signal level at zero heart activity. In applications described in the paper, this signal level is used as zero reference in nonlinear techniques. The method proved to work well and accurately.

Retrograde pulmonary arteriography.

We performed retrograde pulmonary arteriography by means of a pulmonary venous wedge injection in 10 patients with no demonstrable intrapericardial pulmonary arteries by "conventional" angiographic techniques. In all cases but one, the procedure demonstrated the feasibility of a further operation. No complications were observed. Retrograde pulmonary arteriography is an important additional method for determining the existence of surgically accessible pulmonary arteries when other techniques have failed.

Fontan's operation: an expanded horizon.

The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit has now gained a much wider application than initially intended. We have applied this type of operation in a series of 15 patients, five of whom had "classical" tricuspid atresia with concordant arterial connections. The other 10 patients showed a variety of congenital malformations, such as double-inlet univentricular heart with discordant arterial connections, double-outlet right ventricle with common atrioventricular canal. Ebstein's anomaly with almost imperforate tricuspid valve, hypoplasia of right ventricular inflow, and a crisscross heart with straddling right atrioventricular valve. Important associated anomalies, such as common atrium and anomalous pulmonary and/or systemic drainage, required particular adaptation of the technique. Thirteen of the 15 patients survived operation and are presently alive. One death occurred in a patient in whom the preoperative diagnosis had to be adjusted during operation because of unexpected total anomalous pulmonary venous connections. The other death occurred in a patient suffering from the "asplenic syndrome." The autopsy in this patient suggested that the large baffle used for atrial septation and ventricular exclusion had obstructed pulmonary venous return. Our experience thus supports the opinion that Fontan's operation has a place in the treatment of otherwise anatomically uncorrectable lesions.

Respiratory distress due to bronchial compression in persistent truncus arteriosus.

Clinical and pathologic findings are described in a seven-month-old boy who suffered from persistent truncus arteriosus and suddenly developed signs of respiratory distress. Bronchial compression in patients with persistent truncus arteriosus has only rarely been reported.

[The subclavian-pulmonary anastomosis (author's transl)]. / L'anastomosi succlavia-polmonare oggi.

A series of 16 patients who underwent Blalock-Taussig anastomosis for severe cyanosis, without hospital deaths, is reported. Based on this recent, limites experience, and on/an extensive review of the literature the Authors analyze objective criteria to identify the ideal palliative systemic-pulmonary shunt. Early results and late sequelae of Blalock-Taussig, Waterston-Cooley and Potts-Smith anastomosis are compared. It is then proposed that the subclavain-pulmonary anastomosis should be considered the palliation of choice of elective and emergency indication to treat severe cyanosis in those patients for whom corrective surgery can not be contemplated.

Palliative Mustard or Rastelli operation in complete transposition of the great arteries. Option decided by lung biopsy.

A case history is reported of a patient with complete transposition of the great arteries and a ventricular septal defect in whom pulmonary hypertension developed, with a pulmonary resistence of 10 units M.2. Because of the possibility that the pulmonary vascular obstructive disease might have been of recent onset and that a high hematocrit value of 82 percent may have interfered with the precise determination of pulmonary resistance, an open lung biopsy was performed. The histologic findings suggested that the vascular disease was as yet reversible. Because of these findings and the aforementioned considerations, a Rastelli operation was performed instead of a palliative Mustard procedure. At present, 6 months postoperatively, the patient is asymptomatic.

Ebstein's anomaly in persistent common atrioventricualr canal.

This report documents 2 patients who presented with cyanosis early in life. In both instances the necropsy showed the simultaneous occurrence of an imcomplete type of persistent atrioventricular canal and an Ebstein's malformation of the "tricuspid" valve component. In the first patient no clinical investigations were done. In the second patient the electrocardiogram was consistent with persistent atrioventricular canal and the angiocardiogram showed the characteristic goose-neck deformity. Moreover, an abnormality of the right cardiac contour was seen, which in retrospect was correlated with the right-sided Ebstein's malformation. Ebstein's anomaly may significantly alter the clinical and haemodynamic profile of atrioventricular canal and should be considered in atypical cases. The presence of Ebstein's anomaly will complicate surgical repair of the atrioventricular canal.

Congenitally complete heart block. Developmental aspects.

Three cases of congenitally complete heart block are described of hearts in which other minor congenital malformations were not in themselves severe enough to disrupt the atrioventricular conduction system. The cases fitted well into the categorization of complete heart block suggested by Lev. Two exhibited lack of communication between the atrial and conducting tissues, the other had discontinuity of the penetrating atrioventricular bundle. In an attempt to explain why this discontinuity between different segments of the conducting tissues occurs, we re-examined several series of graded human embryos. This investigation suggested that the anulus fibrosus in the normal heart is derived from sulcus tissue of the atrioventricular junction, the endocardial atrioventricular cushions playing a minor role in the separation of atria from ventricles. The relationships between the sulcus tissues and the different components of the atrioventricular junctional area are discussed in terms of an explanation both for the existence of different types of congenitally complete heart block and for persistence of Mahaim (nodo-ventricular and nodo-fascicular) fibers.

Anatomically corrected malposition of great arteries.

Four anomalous hearts are described in which the great arteries arise in unusual fashion from their morphologically appropriate ventricles. This malformation, previously termed anatomically corrected transposition, is now termed anatomically corrected malposition. This is because, following the precedent of Van Praagh and his associates, we now reserve the term 'transposition' to describe the situation in which both great arteries arise from separate morphologically inappropriate ventricles. All the hearts examined exhibited atrioventricular concordance, I with viscero-atrial situs inversus, and 3 with situs solitus. However, there were considerable variations in ventricular morphology between the cases. Thus, 2 cases exhibited atresia of the right atrioventricular valve, and in the remaining 2 cases right and levt ventricular sinuses were both identified. Two of the cases also had pulmonary atresia, and coronary artery anomalies were present in all 4. The cases emphasize the fact that the term anatomically corrected malposition describes not a discrete anomaly but only a ventriculo-arterial relation, which is one of ventriculo-arterial concordance. Doubt has previously been cast upon the existence of this as an anatomical entity. It is concluded that the relation does indeed exist, and furthermore can coexist with all varieties of atrioventricular relations. It is suggested that the differing atrioventricular relations can be distinguished by usage of the terms 'concordant' or 'discordant' anatomically corrected malposition. Finally, it is emphasized that it is necessary to distinguish this anomaly, which in most cases presents with left-sided anterior aorta, from the left-sided anterior aorta more frequently encountered in classically corrected transposition'.

Snapshot diagnosis: syndromes, facies and heart disease.

In some syndromes the facial appearance of the patients is so typical that a correct diagnosis can be made 'à vue'. The five cases presented here are examples of this statement and the additional clinical data make it possible to decide on the syndromes and the complicating cardiac anomalies.

Aberrant origin of left pulmonary artery (vascular sling). Report of the clinical and anatomic features in three patients.

The clinical and anatomical findings in 3 patients with aberrant origin of the left pulmonary artery from the right pulmonary artery ("vascular sling") are presented. All 3 children symptoms of severe respiratory distress shortly after birth. In 2 children the correct diagnosis was suggested from the roentgenogram of the thorax, because of an indentation in the anterior wall of the esophagus. The diagnosis was further endorsed by selective angiography of the aberrant left pulmonary artery. The second patient is of particular interest, since the correct diagnosis was missed because of the presence of a multitude of associated anomalies. These included a tracheobronchial anomaly, a ventricular septal defect with a dilated pulmonary trunk, and a left-ward shift of the heart secondary to pulmonary emphysema on the right. Therefore, the indentation in the esophagus was absent, while the shift and rotation of the heart led to a misinterpretation of the exact course of the left pulmonary artery on the angiocardiogram. These cases are presented to reemphasize that "vascular sling", although rare, is indeed a serious cause of respiratory distress in infancy. Early recognition is of vital importance, since surgical repair of the vascular anomaly seems to be the only benificial procedure in these patients.