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Congenital Absence Complete of the Left Pericardium Heart Block* Report PHILIP VARRIALE, New T a Case Rossi, PI.INIo M,D.,** of York, and M.D.f W. J. AND New GRACE, M.D., F.C.C.P4 York palpable. The rhythm was regular. A grade II/VI ejection systolic murmur was audible along the left sternal border with maximal intensity in the second and third intercostal space. Splitting of the second sound occurred on inspiration. There was slight accentuation of the pulmonic was HE RECOGNITION AND DEFINITIVE nosis of absent pericardium impetus within recent years. described for many years anomaly found tion, it was reported not the at post-mortem 1959 case of the pericardium was successfully of with concomitant picted with proper 15.7 mg pericardium white tial count in terms of diagnosis, and the and The ventricular cardiogram with was an showed cardiogram also block done normal cardiac to 1). the left of The atrioventricular heart was showed abnormal prominence (Fig. urea all within displaced apparent contour complete were film an and of and Differen- cent; per blood sugar x-ray fingers hemoglobin 9,500/mm3. blood heart the strong. Urinalysis, Chest with a of 46 of vascularity hemithorax treat- count fasting limits. contour. left showed normal. on abdominal of were hematocrit cell and normal and paris stressed prognosis pulses was pulmonary underscored. The differentiation of complete tial deficiency of the pericardium Peripheral cent; blood nitrogen deand is presented techniques clubbing studies per palpable was Moderate Laboratory block radiologic, studies diagnostic present. was the diagnosis by diagnostic heart organ examination. absence left angiographic, No component. et al’ life. deficient complete hemodynamic Ellis of congenital during case examinathat in which established pneumothorax This has gained Although well as a curious until first DIAG- the electro- dissociation (Fig. (Fig. 2). A vector- 3). ment. Right CASE This St. 29-year-old Vincent’s ical white Hospital evaluation and heart hemodynamics REPORT: man on of an 29, abnormal electrocardiogram admitted was April 1964 for chest noted x-ray during a catheterization (Table showed 1). Selective normal angiography to med- #{149} film routine ‘! Li examination. He was the age the Armed sumably 16 and, years Forces that this was The symptoms. failed at to examination, cardiac in good past murmur later, physical of he a heart of two because stated no first informed of pre- murmur. health medical pass He and history offered was nega- Hg, pulse tive. The blood was 60 per ture cyanosis were or flat clear. the in lary sixth line eFrom diology, ter of intercostal produced the Departments St. New distress. Vincent’s Cervical position. apical and and temperadeveloped, with well was recumbent cardiac mm 130/88 regular, respiratory the in The was and He normal. was no pressure minute impulse space was and a moderate of Hospital Lungs were palpated anterior axil- lift. No Medicine veins thrill and and Medical RaSignificant features include leftward of the heart without tracheal deviaaddition, three distinct convexities are noted along the left border of the heart. These are the aortic knob, pulmonary artery segment and the left ventricular contour. Cen- FIGURE York. **Assistant Attending, Department of Medicine. of Special Procedures, Department of diology. Director, Department of Medicine. fChief 1: displacement tion. In Ra- 405 Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 406 VARRIALE, : 11 ROSSI AND 11.1. II#{149}..I.l 2: the Conventional The ventricular leads occurs block. precordial was performed displaced to right anterior ventricle half flow tract artery of were posterior the AP 4). left of appeared the the of the (Fig. the normal. right lead rate between The the I (junctional V, and tricuspid shows atnioventricular ir 48 per and was tion cardium and occupied shadow. The out- left ventricle was valve the The transitional film. Hence, technique silhouette in posi- air and He well. complete complex of was was pen- on chest studied, with 500 left pleural noted absent findings patient the using between and the pericardium (Fig. and of ml cavity asymptomatic parietal discharged was of the pneumothorax, into of visceral by the of collection possibility The suggested air introduced occupied in origin. was x-ray the pulmonary normal with QRS dissociation minute. V,. The and aorta rhythm) spine. of the cardiac The i electrocardiogram ventricle The L.Ii:’I1:I1i vertebral large cardiac portion projection. 12 of the Chest I1r I -I FIGURE heart Diseases GRACE a right 5). has been F DISCUSSION H Congenital pericardial dominantly complete left-sided. deficiency or as a partial left atrium.’ The ascribed LS left to defect, pulmonary defects They of the usually artery left-sided premature predilection the or left has been development of the membrane, atrophy of pre- overlying segment, to incomplete pleuropericardial are present as either left pericardium, the secondary left duct of Cuvier.1 Strict 3: The horizontal plane shows a CCW with posterior displacement of the maximum vector. A prominent S loop is present. The loop in the frontal plane is CCW. The initial portion is to the left and slightly inferior and terminal activation occurs toward the right. The left saggital planar projection depicts .a CCW loop with predominant posterior displacement. (The direction of inscription is denoted by the sharp end of the tear-drop). FIGURE loop QRS QRS tween complete differentiation must partial pericardial variety. In the be made be- defect and the latter, the pericar- dial and pleural space form a common cavity and the heart, displaced toward the left, assumes an abnormal anatomic position within the chest. In ciency, the heart maintains Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 partial pericardial defi- its normal posi- Volume 52, September. is also ventricle tion CONGENITAL The tricuspid displaced to the is also noted. in the cardial ings of 3 4A: FIGURE ity No. 1967 a chest cavity. may be left hilar left valve and and resides Here the either ABSENCE is displaced extends to within chest normal, mass or the x-ray or apparent OF to the left the outermost LEFT of PERICARDIUM the vertebral contour of pen- ment of find- there is herniation suggestive enlarge- pendage In the the 407 spine. heart. pulmonary case, artery of through this The right Enlargement the the left ventricular of the cavright segment if atrial ap- of the left defect.3’4 complete absence -t-. FIGURE 4C 4BFIGURE FIGURE 4 B and C: The interventricular septum assumes a parallel body. The right ventricle occupies the anterior half of the heart, and This is a consequence of an absent pericardium which causes cardiac (pseudo-levorotation). course with the the left ventricle, rotation to the Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 frontal plane of the the posterior half. left and posteriorly 408 VARRIALE, TABLE 1-DATA Pressures OF in mm Right atrium Right ventricle Pulmonary CARDIAC 31/5 26/9 artery (12) left (17) Content Rest artery Brachial artery Exercise 12.58 12.12 18.47 18.54 saturation oxyhemoglobin blood 94.3% liters/mm blood Systemic Stroke rate volume Hydrogen Dye uptake dilution 2.61 50 56 95 ml. was findings on chest most striking feature FIGUGE 5A: Diagnostic the right was right parietal ing by two roentgenograms. related pneumothorax: penicardium to signifiThe an abnor- Patient (arrow). Another diagnosis the lying in The heart shows along however, pneumothorax. air into a distinct left visceral chest is the left lateral border. separating the pericardium. to the demaccord- right posterior roentgenograms. In this decubitus displacement Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 ob- This with the patient lying a horizontally directed supine. further the left pneumo- cardiac air most favorable pneumopericardium, et al,’ the aortic artery segment contour. technique that permits is the lateral roentgenognam patient the the and be performed left side, with suggested of a three confirmed, as of the with of the of left discernable to Ellis lique was along This noted pulmonary ventricular 500 ml produced The position onstration of 85 ml. studies-normal cant outlines 2.17 studies-normal penicardium cardium position. 5.15 flow L/min/M’ Ventricular 4.27 left left, Secondly, consisting utilization of space the were diagnosis after to position. border, pericardium flow heart convexities Injection pleural Arterial Systemic the cardiac only in Per Cent Volumes of tracheal The Oxygen the Chestof knob, elongated and flattened (10)* Pressure Pulmonary shift distinct 29/9 (8) Pulmonarywedge *Mean 5 28/5 Diseases GRACE normal Exercise 5 AND mal CATHETERIZATION Rest Hg ROSSI position, position. to the can on the beam. accurate with the heart Pneumoperileft in this Volume No. 52, September, 3 CONGENITAL 1967 is displaced posteriorly the left pericardium mothorax is study if the of the an against is deficient. diagnosis of the ing the heart, of complete on definitive cardium. This diagnosis added technique, with of have cluding with of congenital congenital has Despite not Stokes may be the pre- disease, been presence patient There attack in- broncho- pulmonary lobe.4 kind of conduction previously was described or these combined completely no been described and one history dyspnea. asympof Most absence and defects been described. left pain pectoris is death a serious fall through a mechanical reported, as sequelae Adams- gical patients the be absence of partial other herniation of left pen- ominous argue would or to oc- the Complete A activity expected possible defect hand, strangula- of of cardiac not correction.#{176} sudden foramen.’#{176}” These exists. and of with pericardial if complete memay output cases a result would cardium to activity in cardiac Two restriction of leading been strangulation in have incarceration heart left exists and of cardiac syncope. com- the pericarpotential of always appendage produce the more pericardium Transient have to indistinguishable absence of the frightful atnial of have pain, related perhaps of the restriction tion cases chest was sudden chanical death pericardium position.”3 with partial Moreover, small pain chest mon the the body the unexplained which angina syncope of Several with in in dium.3 409 asymptomatic. from cur of was been subsequent deficiency. the our tomatic. peri- anomalies, heart pericardial lesions, absent this case was heart block, genic cysts, and aberrant To our knowledge, this disorder in promot- congenital origin. Pericardial been described in association a variety complete have Recurrent change however, PERICARDIUM with hand, deficiency, particularly appendage herniates border.3’4”3”4 interest in of complete association sumably defects of LEFT changes other of no value conclusive in partial when the left atrial beyond the pericardial Of deficiency positional was of substantiated. the abnormal but a method is to be Angiocardiography, delineated the spine if Left pneu- indispensable pericardium OF ABSENCE for its sur- deficiency, not require on surgical intervention. Cardiac parent enlargement than the left the leftwai-d real, Clinically, a may complete have influenced be myocardial the of of heart. manifested heart block actual increased increased absence beyond slow heart ap- consequence of impulse The with more complete as this apical The be displacement boundary.’3 sent with pericardium forceful of the sequent may as its rate a normal associated in this case enlargement may by dint stroke volume, with subdiastolic length of the fibers. life span of most pericardium compromised. The pericarditis as genital defect mately 75 patients is probably per with not occurrence ab- seriously of pleuro- a complication of this has in approxi- cent been found of cases.’1 con- REFERENCES FIGURE Posterior retrosternal 5B: Translateral displacement space. supine of the view heart of and the chest. increased 1 ELLIS, K., “Congenital cardium,” N. E. AND HIMMELSTEIN, deficiencies in the parietal Am. J. Roent., 82:125, 1959. LEEDS, Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 A.: pen- VARRIALE, 410 2 3 4 5 6 7 8 ROSSI PERNA, G.: “Sopra un arresto di sviluppo della sierosa penicardica dell’uomo.” Anat. Anz., 35: 323, 1909-1910. TUCKER, D. H., MILLER, D. E. AND JACOBY, W. J.: “Congenital partial absence of the pencardium with henniation of the left atrial appendage,” Am. J. Med., 35:560, 1963. BAKER, W. P., SCHLONG, H. A. AND BELLENGER, F. P.: “Congenital partial absence of the pericardium,” Am. J. Cardiol., 16:133, 1965. KAVANOGH-GRAY, D., MUSGROVE, E. AND STANWOOD, D.: “Congenital penicardial defects,” New Engi. J. Med., 265:692, 1961. RUSBY, N. L. AND SELLORS, T. H.: “Congenital deficiency of pericardium associated with bronchogenic cyst,” Brit. J. Surg., 32:357, 1945. Osooor, R. AND SPECTOR, B.: “Defective pencardial sac and inter-atnial septum and atresia of pulmonic orifice,” Am. J. Di:. Children, 61:1028, 1941. JONES, P. H.: “Developmental defects in lungs,” Thorax, 10:205, 1955. C0,-O, The original phy has been contrast subfaclal of the after planes of mediastlnum demonstration of of the CO, medlastinum. are then the to 0, as dissect the Polytomograms for detailed made Instances been of resulted per- and respiratory refiux. symptomatic tract in of patients proved 87.5 per 13 14 surgical therapy for surgery resistant in include to in patients medical Cardiovasc. “Incomplete Surg., pericardial into left 28:209, pleural 1887. S. AND “Congenital penicardial 6:167, 1944. SOUTHWORTH, H. “Congenital defects sac: cavity,” Tr. AND Brit. STEVENSON, of pericardium,” J.: R. Heart WRIGHT-SMITH, defects,” es- Obst. J., C. Arch. S.: mt. Med., 61:223, 1938. FOWLER, N. 0.: “Congenital defect of the pericardium,” Circulation, 26:114, 1962. ROGGE, J. D., MISHKIN, M. E. AND GENOVESE, P. D.: “Congenital partial penicardial defect of herniation of the left atnial appendage,” Ann. mt. Med., 64:137, 1966. For reprints, please write: 11th Street, New York City Dr. Grace, 10011. 153 West POLYTOMOGRAPHY cent technically successful examinations with no significant morbidity or mortality. It is emphasized that pathologic processes, grossly similar but histologically dissimilar, cannot be differentiated by method. S., A. P. M. ilcINs, 88:519, AND bronchogenic restoring with clinically URSCHEL, HIATAL F.: #{149}C0-O for the pre- Radiology, carcinoma.” HERNIA who demonstrate Reconstruction on the technique provement 53:21, of W. BUGDEN, polytomography 1967. ment refiux. based refiux AND with evaluation diagnosis treat- and SUNDERLAND, REFLUX Cinefluorography useful R.: heart London, operative secondary to cardioesophaor without hiatal hernia. the etiology of esophageal complications. has Indications 12 Thorac. BOXALL, cape of Soc., 11 AND BOLL, of the pen- deficiency 1960. pneumomediastinography suspected GASTROESOPHAGEAL Gastroesophageal refiux geal incompetence, with is a significant factor in of the esophagus and evaluation 10 BERNE, has J. 40:49, J. S. C., WILSON, “Congenital JR.: candium,” this anatomy. CO,-O2 pneumomedlastinography formed in this fashion in 64 bronchogenle carcinoma. This A. 9 HERING, R. E., WITH CO, pneumomediastlnograby the Injection of injecting Diseases of the Chest GRACE PNEUMOMEDIASTINOGRAPHY technic modified material AND over the cardloesophageal H. C. AND and hiatai of significant gastroesophageal the gastroesophageal angle of Belsey is a marked im- modified PAULSON, hernia. 1967. Downloaded From: http://publications.chestnet.org/pdfaccess.ashx?url=/data/journals/chest/21453/ on 05/03/2017 1. Allison competence. D. L.: Thor. and technique in “Gastroesophageal Cardiovaic. Surg.,