“Masked” pulmonary venous obstruction in patients with isomerism of the right atrial appendages: an overstated association

2002 ◽  
Vol 12 (2) ◽  
pp. 113-118 ◽  
Author(s):  
C. Becket Mahnke ◽  
George G. Sandor ◽  
Gerard J. Boyle ◽  
Steven A. Webber

Isomerism of the right atrial appendages is associated with anomalies of pulmonary venous return, which may be obstructive. The associated pulmonary arterial obstruction, however, has been reported to “mask” the pulmonary venous obstruction, with resultant pulmonary edema following augmentation of the flow of blood to the lungs. We postulated that the frequency of “masked” pulmonary venous obstruction has been overreported in the literature. To ascertain the true situation, we evaluated the frequency of “masked” pulmonary venous obstruction in a large, unselected, group of patients with isomerism of the right atrial appendages. We evaluated the clinical, anatomic, and radiographic data of 65 newborns seen with this syndrome since 1970. Development of pulmonary venous obstruction, both before and after systemic-to-pulmonary shunting and/or infusion of prostaglandin E1, was determined and correlated to clinical and radiographic findings. Of the 65 patients, 19 (29%) were ultimately diagnosed with pulmonary venous obstruction. The pulmonary veins themselves connected in infradiaphragmatic fashion in 10 patients, supracardiac in 3, to the atriums directly in 1, and in mixed fashion in the other 5. Pulmonary venous obstruction was readily apparent in 15 of the 19, as demonstrated by pulmonary edema on initial chest radiography. The remaining four cases ultimately diagnosed with pulmonary venous obstruction received augmentation of pulmonary blood flow with resultant pulmonary edema. Of these four severely cyanosed patients, pulmonary vascular markings on the initial chest radiograph had been normal in one but increased in three. Due to the presence of these clinical markers, they do not truly represent “masked” pulmonary venous obstruction. Except for two patients with minimally obstructed pulmonary arterial blood flow, 44 patients without pulmonary venous obstruction had normal or decreased pulmonary vascular markings at presentation. Of these 44, 14 received infusions of prostaglandin E1, with none developing pulmonary edema. We conclude that pulmonary venous obstruction is usually readily apparent at time of presentation in patients with right isomerism, and that “masked” pulmonary venous is a very rare event which has been over-emphasized in the literature. Careful evaluation of clinical and radiographic findings at time of presentation can correctly identify pulmonary venous obstruction in such patients.

2005 ◽  
Vol 35 (6) ◽  
pp. 580-586 ◽  
Author(s):  
Kevin S. Roman ◽  
Christian J. Kellenberger ◽  
Christopher K. Macgowan ◽  
John Coles ◽  
Andrew N. Redington ◽  
...  

1992 ◽  
Vol 2 (1) ◽  
pp. 95-99 ◽  
Author(s):  
Ing-Sh Chiu ◽  
Nan-Koong Wang ◽  
Mei-Hwan Wu ◽  
Fen-Fen Wu ◽  
Chi-Ren Hung

SummaryObstruction to the pulmonary venous return is a frequent associated anomaly in patients with isomerism of the right atrial appendages. Yet, preoperative diagnosis by means of either cross-sectional echocardiography or cardiac catheterization can be intriguing. Indeed, the presence of two morphologically right lungs reduce considerably the size of window for precordial echocardiography. Also, in the presence of severe pulmonary stenosis or atresia, it can be difficult at cardiac catheterization to enter the pulmonary trunk. In these patients, construction of a systemic-to-pulmonary artery anastomosis will almost inevitably result in pulmonary edema. Between May 1984 and December 1988, five patients with isomerism of the right atrial appendages, severely decreased pulmonary blood flow and concealed obstruction to the pulmonary venous return were admitted to our hospital. A modified Blalock Taussig shunt by interposition of a polytetrafluoroethylene prosthesis was performed in each patients and all of them developed pulmonary edema. Three patients died despite appropriate medical treatment. The remaining two patients were successfully treated by banding of the Blalock shunt. This was performed in the first patient at the time of the initial surgery, when prior to closure of chest, pulmonary edema became manifest. The second patient who developed pulmonary edema early postoperatively, underwent cardiac catheterization to confirm the clinical diagnosis of obstruction to the pulmonary venous return. Reduction of blood flow through the Blalock shunt with resolution of edema was initially achieved by means of a partially occluding balloon catheter. Pulmonary edema recurred one week later because of rupture of the balloon and the patient eventually underwent a successful banding of the Blalock shunt through a left thoracotomy. We conclude that preoperative assessment of the pulmonary venous return is mandatory in patients with right isomerism and reduced pulmonary blood flow. Construction of a modified Blalock-Taussig shunt in the presence of concealed obstruction to the pulmonary venous return will almost inevitably cause pulmonary edema. Banding of the Blalock shunt can be successful, as observed in our experience, for the management of this serious complication.


1985 ◽  
Vol 58 (4) ◽  
pp. 1092-1098 ◽  
Author(s):  
M. D. Walkenstein ◽  
B. T. Peterson ◽  
J. E. Gerber ◽  
R. W. Hyde

Histological studies provide evidence that the bronchial veins are a site of leakage in histamine-induced pulmonary edema, but the physiological importance of this finding is not known. To determine if a lung perfused by only the bronchial arteries could develop pulmonary edema, we infused histamine for 2 h in anesthetized sheep with no pulmonary arterial blood flow to the right lung. In control sheep the postmortem extravascular lung water volume (EVLW) in both the right (occluded) and left (perfused) lung was 3.7 +/- 0.4 ml X g dry lung wt-1. Following histamine infusion, EVLW increased to 4.4 +/- 0.7 ml X g dry lung wt-1 in the right (occluded) lung (P less than 0.01) and to 5.3 +/- 1.0 ml X g dry wt-1 in the left (perfused) lung (P less than 0.01). Biopsies from the right (occluded) lungs scored for the presence of edema showed a significantly higher score in the lungs that received histamine (P less than 0.02). Some leakage from the pulmonary circulation of the right lung, perfused via anastomoses from the bronchial circulation, cannot be excluded but should be modest considering the low pressures in the pulmonary circulation following occlusion of the right pulmonary artery. These data show that perfusion via the pulmonary arteries is not a requirement for the production of histamine-induced pulmonary edema.


2011 ◽  
pp. 55-62
Author(s):  
James R. Munis

What does right atrial pressure (PRA) do to cardiac output (CO)? On the one hand, we've been taught that PRA represents preload for the right ventricle. That is, the higher the PRA, the greater the right ventricular output (and, therefore, CO). This is simply an application of Starling's law to the right side of the heart. On the other hand, we've been taught that PRA represents the downstream impedance to venous return (VR) from the periphery. That is, the higher the PRA, the lower the VR, and therefore, the lower the CO. The point of intersection between the 2 curves defines a unique blood flow rate, which is both CO and VR at the same time.


2009 ◽  
Vol 19 (6) ◽  
pp. 594-600 ◽  
Author(s):  
Nicodème Sinzobahamvya ◽  
Claudia Arenz ◽  
Julia Reckers ◽  
Joachim Photiadis ◽  
Peter Murin ◽  
...  

AbstractTotally anomalous pulmonary venous connection, when also associated with a functionally univentricular connection, is known to have a poor outcome. We retrospectively analysed results for 19 patients undergoing surgery for this combination of lesions between 1995 and February 2009.Of the patients, 12 were neonates, with 11 presenting with signs of pulmonary venous obstruction. In 3 patients, a modified Blalock-Taussig shunt had been constructed. The dominant ventricle was of right ventricular morphology in 17 of the 19 patients, and double inlet was present in all bar 1. Pulmonary atresia or stenosis was found in 14 patients, a common atrioventricular junction in 14 patients, and isomerism of the right atrial appendages in 12 patients, respectively. Comprehensive Aristotle scores ranged from 14 to 23.50. The mean was 16.55, with a standard deviation of 2.19. Pulmonary venous rerouting was combined in 6 patients with construction of a modified Blalock-Taussig, in 4 with banding of the pulmonary trunk, in another 4 with a bidirectional Glenn anastomosis, in 3 with creation of a total cavo-pulmonary connection, and in 1 each with enlargement of the right ventricular outflow tract and the Norwood procedure. Of the cohort, 8 patients died early due to pulmonary hypertension, with all patients having Aristotle scores of at least 18 points dying. Among the 11 early survivors, 5 needed mechanical ventilation for over a week, but 5 patients died later, 3 due to pulmonary hypertension and 2 due to infection. Actuarial survival stabilized at 31.6%, with standard error of 10.7%, from one year onwards. Conversion to the Fontan circulation was successful in 5 patients.Our experience confirms that totally anomalous pulmonary venous connection, when associated with the functionally univentricular arrangement, carries one of the worst outcomes in current surgical practice. Use of the Aristotle comprehensive complexity scores effectively discriminates those patients with this condition at particularly high risk.


2006 ◽  
Vol 108 (3) ◽  
pp. 418-421 ◽  
Author(s):  
Christina Routsi ◽  
Christos Charitos ◽  
Demetra Rontogianni ◽  
Zoe Daniil ◽  
Epaminondas Zakynthinos

2006 ◽  
Vol 291 (6) ◽  
pp. H3114-H3121 ◽  
Author(s):  
Yichun Sun ◽  
Israel Belenkie ◽  
Jiun-Jr Wang ◽  
John V. Tyberg

Diastolic suction (DS) can be defined as that property of the ventricle by means of which it tends to refill itself during early diastole, independent of any force from the atrium. Although thought to be significant in the left ventricle (LV), DS in the right ventricle (RV) has received little attention, probably because of RV geometry. Our recent LV studies have shown that DS is related to both decreased elastance (i.e., τ, the relaxation time constant) and end-systolic volume (VLVES), thus reconciling the two mechanisms that have been used to explain the concept of DS. We hypothesized that RV DS would similarly depend on τ and VRVES. In six anesthetized open-chest dogs, aortic, RV, right atrial (RA), pulmonary arterial (PA), and RV pericardial pressure, tricuspid velocity, and PA flow were measured. VRVES was calculated by measuring distances between eight ultrasonic crystals. An empirical index of relaxation, τ′, and VRVES were manipulated by volume loading/caval constriction and isoproterenol/esmolol. We calculated the total energy (IW−) of the backward expansion wave generated during RV relaxation and that component causing DS [IW−(DS)]; i.e., the energy remaining after tricuspid valve opening. IW− [IW−(DS) also] was found to be inversely related to τ′ and to VRVES {i.e., IW− = −8.85· e(−0.0423τ′)· e[−0.0665(%VRVES)]}. Thus, as for the LV, the energy of the backward-going wave generated by the RV during relaxation depends on both the rate at which elastance decreases and the completeness of ejection. Despite the thin wall and nonspherical shape of the RV, DS appears to be an important mechanism.


1994 ◽  
Vol 73 (11) ◽  
pp. 829-831 ◽  
Author(s):  
Jou-Kou Wang ◽  
Hung-Chi Lue ◽  
Ing-Sh Chiu ◽  
Mei-Hwan Wu ◽  
Chung-I Chang

1984 ◽  
Vol 56 (5) ◽  
pp. 1418-1422 ◽  
Author(s):  
J. F. Green ◽  
N. D. Schmidt

Increases in pulmonary blood flow can elicit hyperpnea. To examine the mechanisms responsible, we surgically isolated the systemic and pulmonary circulations in six dogs and independently controlled PCO2, PO2, and blood flow in each circuit. Anesthesia was induced with ketamine and maintained with halothane. Systemic venous return was drained from the right atrium and passed through a membrane oxygenator and heat exchanger; blood was returned to the ascending aorta (Qs). An identical bypass was established for the pulmonary circulation, draining blood from the left atrium and returning it to the pulmonary artery (Qp). The lungs were initially ventilated with room air. Qs and systemic arterial CO2 gas tension were maintained at approximately 0.080 1 X min-1 X kg-1 and 40 Torr, respectively. Pulmonary arterial CO2 gas tension was set near 55 Torr, and Qp varied. Ventilatory drive was assessed by minute integration (MI) of the activity recorded from the central end of the left C5 root of the phrenic nerve. MI increased as much as 160% above control as Qp was increased over the range of 0.025 (control) to 0.175 1 X min-1 X kg-1. When pulmonary CO2 gradients were eliminated by a rebreathing technique, MI was independent of Qp. These results suggest that CO2-sensitive pulmonary receptors respond to the change in pulmonary PCO2 gradients which occur when Qp is elevated (breathing room air) augmenting ventilation.


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Mathias Zuercher ◽  
Ronald W Hilwig ◽  
Jon Nysaether ◽  
Vinay M Nadkarni ◽  
Marc D Berg ◽  
...  

Background : Incomplete chest recoil during cardiopulmonary resuscitation (CPR) (ie, leaning on the chest during the decompression phase) is purported to decrease venous return, and thereby decrease forward blood flow. Aim To determine the effect of 10% and 20% lean on hemodynamics during piglet CPR. Methods : 10 piglets (10.7±1.2 kg) were anesthetized with isoflurane and instrumented with micromanometer-tipped catheters in the right atrium (RA) and aorta (Ao). After induction of ventricular fibrillation, CPR was provided in three-minute epochs with no lean, 10% lean, or 20% lean while aortic systolic pressure (AoS) was targeted at 80–90 mmHg. Because the mean force to attain 80 –90 mm Hg AoS was 18 kg in preliminary studies, 10% and 20% lean were provided as 1.8 and 3.6 kg weights on the chest, respectively. Left ventricular myocardial blood flow (MBF) and cardiac index (CI) were determined by fluorescent, color-microsphere technique. Statistics: paired t -test and repeated measurement ANOVA for parametric, Wilcoxon Rank Sum Test and Friedman’s ANOVA for non-parametric data. Results : 10% and 20% lean resulted in higher right atrial diastolic pressure (RAD) and lower coronary perfusion pressure (CPP) than no lean. Hemodynamics were not different with 10% lean vs. 20% lean. Mean 10%–20% lean resulted in substantially lower MBF and CI than no lean (Table ). Conclusions : 10–20% leaning during CPR increases RAD, decreases CPP, and substantially decreases MBF and CI. Table


Sign in / Sign up

Export Citation Format

Share Document