scholarly journals CLOSING OF INTERVENTRICULAR SEPTUM DEFECT IN ONE-STEP AND STEPWISE SURGICAL TREATMENT IN PATIENTS WITH PULMONARY ATRESIA AND AORTO-PULMONARY COLLATERALS

2015 ◽  
Vol 174 (4) ◽  
pp. 9-12
Author(s):  
A. A. Morozov ◽  
R. R. Movsesyan ◽  
V. G. Lyubomudrov
Keyword(s):  
Blood Flow ◽  
Surgical Treatment ◽  
Pulmonary Blood Flow ◽  
Interventricular Septum ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Definitive Repair ◽  
One Step ◽  
Pulmonary Arterial ◽  
The Right

Pulmonary atresia with defect of interventricular septum and collateral pulmonary blood flow refers to complicated congenital malformation of the heart. Surgical treatment represents itself as very difficult task because of anatomical variability of this abnormality. The main problem of surgery is a definitive repair of the defect including correction of maldistributions of pulmonary arterial bed (unifocalization of pulmonary blood flow), reconstruction of outflow tract of the right ventricle and closing of interventricular septum defect. The performance of closing interventricular septum defect could be successful in patients with pulmonary atresia and collateral pulmonary blood flow in case of stepwise and one-step surgical treatment. The combination of maximal number of pulmonary segments and sufficient development of central pulmonary arteries facilitates to progress of assigned task. Patients with the level of pulmonary-arterial index more than 170 mm/m² and integrity of pulmonary arterial bed, centralization of pulmonary segments (minimum 15) could be considered as a candidate for definite repair of the defect.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

Determinants for outcome of hypoplastic right ventricle with duct-dependent pulmonary blood flow presenting in the neonatal period

1992 ◽  
Vol 2 (4) ◽  
pp. 377-381 ◽  
Author(s):  
Michael L. Rigby ◽  
Micelia Salgado ◽  
Celia Silva
Keyword(s):  
Blood Flow ◽  
Right Ventricle ◽  
Pulmonary Blood Flow ◽  
Pulmonary Valve ◽  
Pulmonary Atresia ◽  
Cross Sectional ◽  
Mitral Valves ◽  
Significant Difference ◽  
The Right ◽  
Pulmonary Valvar Stenosis

SummaryA retrospective study was undertaken of patients with hypoplastic right ventricles, either with pulmonary atresia and intact ventricular septum or critical pulmonary valvar stenosis, and duct-dependent pulmonary blood flow who were investigated at the Royal Brompton Hospital between January 1976 and December 1990. The diagnosis was made on the basis of at least one diagnostic method (cross-sectional echocardiography, cardiac catheterization and angiography, or autopsy). Of the patients, 56 (82%) were found to have an imperforate pulmonary valve or infundibulum (pulmonary atresia), while 12(18%) had critical pulmonary valvar stenosis. The ratio of the diameters of the tricuspid and mitral valvar orifices was measured angiographically during diastole, and the right ventricle was analyzed according to the presence or overgrowth of the inlet, apical trabecular and outlet components. A correlation was made between the severity of the disease and the outcome. The overall mortality was 53% when those not undergoing any surgery were excluded, and significant differences were found between the group dying and those who survived. The incremental risk factors for death were a ratio between the diameter of the tricuspid and mitral valves of less than 0.6; the presence of fistulous communications with the coronary arteries; and obliteration of the apical trabecular component of the right ventricle. There was no significant difference between the group with pulmonary valvar atresia and the group with critical stenosis of the pulmonary valve.

Mechanism of hyperpnea induced by changes in pulmonary blood flow

1984 ◽  
Vol 56 (5) ◽  
pp. 1418-1422 ◽  
Author(s):  
J. F. Green ◽  
N. D. Schmidt
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Venous Return ◽  
Membrane Oxygenator ◽  
Co2 Gas ◽  
Ventilatory Drive ◽  
Systemic Venous Return ◽  
Pulmonary Arterial ◽  
The Right ◽  
Breathing Room

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.

A Hemi Fontan Operation Performed by an Engineer: Considerations on Virtual Surgery

2013 ◽  
Author(s):  
Giovanni Biglino ◽  
Ethan Kung ◽  
Adam Dorfman ◽  
Andrew M. Taylor ◽  
Edward Bove ◽  
...  
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Fontan Operation ◽  
Hybrid Approach ◽  
Pulmonary Arteries ◽  
Virtual Surgery ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
The Right ◽  
Cavopulmonary Connection

Single ventricle circulation, characterized at birth by a rudimentary or absent left or right ventricle, presents a challenging and life-threatening physiological scenario. Surgical palliation aims to restore the balance between systemic and pulmonary blood flow and is staged, each of the three stages presenting the surgeon with different options: - Stage 1 (Norwood procedure) involves different types of shunting to source pulmonary blood flow, or recently a hybrid approach [1]; - Stage 2 can involve a superior cavopulmonary connection (Glenn operation) or patching between the right atrium and the pulmonary arteries (Hemi Fontan operation [2]); - Stage 3 involves a total cavopulmonary connection with extracardiac conduit or lateral tunnel, or with novel alternatives such as the Y-graft [3].

TMEM16A Regulates Pulmonary Arterial Smooth Muscle Cells Proliferation via p38MAPK/ERK Pathway in High Pulmonary Blood Flow-Induced Pulmonary Arterial Hypertension

2020 ◽  
Vol 58 (1) ◽  
pp. 27-37
Author(s):  
Dongli Liu ◽  
Kai Wang ◽  
Danyan Su ◽  
Yanyun Huang ◽  
Lifeng Shang ◽  
...  
Keyword(s):  
Blood Flow ◽  
Smooth Muscle ◽  
Vascular Remodeling ◽  
Pulmonary Blood Flow ◽  
Pulmonary Arteries ◽  
Erk Signaling ◽  
Shunt Group ◽  
Pulmonary Arterial ◽  
Arterial Smooth Muscle Cells ◽  
Pulmonary Arterial Smooth Muscle

<b><i>Objective:</i></b> Pulmonary arterial hypertension (PAH) is a complex disease of the small pulmonary arteries that is mainly characterized by vascular remodeling. It has been demonstrated that excessive proliferation of pulmonary arterial smooth muscle cells (PASMCs) plays a pivotal role in vascular remodeling during PAH. The present study was undertaken to explore the role of TMEM16A in regulating PASMCs proliferation in high pulmonary blood flow-induced PAH. <b><i>Methods:</i></b> Aortocaval shunt surgery was undertaken to establish an animal model. Pulmonary artery pressure and pulmonary vascular structure remodeling (PVSR) were tested. Immunohistochemical staining and Western blot were performed to investigate the expression of TMEM16A. The proliferation of PASMCs was tested by the MTT assay. After treating PASMCs with TMEM16A-siRNA, the expression of proliferating cell nuclear antigen (PCNA), phosphorylated p38 mitogen-activated protein kinase (p-p38MAPK), and phosphorylated extracellular signal-regulated kinase (p-ERK) signaling in PASMCs were tested. <b><i>Results:</i></b> PAH and PVSR developed 11 weeks postoperation. Elevated expression of TMEM16A accompanied by high expression of PCNA in pulmonary arteries of the shunt group was observed. The increased proliferation of PASMCs and increased expression of TMEM16A and PCNA, along with activated p-p38MAPK and p-ERK signaling in PASMCs of the shunt group, were all attenuated by siRNA-specific TMEM16A knockdown. <b><i>Conclusion:</i></b> TMEM16A regulates PASMCs proliferation in high pulmonary blood flow-induced PAH, and the p38MAPK/ERK signaling pathway is probably involved.

Tetralogy of Fallot With Pulmonary Atresia: Anatomy, Physiology, Imaging, and Perioperative Management

2020 ◽  
pp. 108925322092048
Author(s):  
Madhusudan Ganigara ◽  
Eyal Sagiv ◽  
Sujatha Buddhe ◽  
Aarti Bhat ◽  
Sathish M. Chikkabyrappa
Keyword(s):  
Blood Flow ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Ductus Arteriosus ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Sole Source ◽  
Aortic Insufficiency ◽  
Collateral Arteries ◽  
Antegrade Flow

Tetralogy of Fallot (ToF) with pulmonary atresia (ToF-PA) is a complex congenital heart defect at the extreme end of the spectrum of ToF, with no antegrade flow into the pulmonary arteries. Patients differ with regard to the sources of pulmonary blood flow. In the milder spectrum of disease, there are confluent branch pulmonary arteries fed by ductus arteriosus. In more severe cases, however, the ductus arteriosus is absent, and the sole source of pulmonary blood flow is via major aortopulmonary collateral arteries (MAPCAs). The variability in the origin, size, number, and clinical course of these MAPCAs adds to the complexity of these patients. Currently, the goal of management is to establish pulmonary blood flow from the right ventricle (RV) with RV pressures that are ideally less than half of the systemic pressure to allow for closure of the ventricular septal defect. In the long term, patients with ToF-PA are at higher risk for reinterventions to address pulmonary arterial or RV-pulmonary artery conduit stenosis, progressive aortic root dilation and aortic insufficiency, and late mortality than those with less severe forms of ToF.

Transposition of the great arteries {S,D,D} and absent proximal left pulmonary artery

1995 ◽  
Vol 5 (2) ◽  
pp. 199-201
Author(s):  
Dipak Kholwadwala ◽  
Vincent A. Parnell ◽  
Rubin S. Cooper
Keyword(s):  
Blood Flow ◽  
Pulmonary Artery ◽  
Pulmonary Blood Flow ◽  
Left Ventricular Outflow Tract ◽  
Pulmonary Arteries ◽  
Left Pulmonary Artery ◽  
Ventricular Outflow Tract ◽  
Left Ventricular ◽  
Left Ventricular Outflow ◽  
The Right

while preferential blood flow to the rightpulmonary artery has been described in transposition of the great arteries with or without obstruction of the left ventricular outflow tract, this disparity of pulmonary blood flow is not present in newborns.1We report a newborn with transposition in whom there was discontinuity of the pulmonary arteries and ductal blood supply to the left pulmonary artery. To our knowledge, this entity has not been described in newborns with transposition of the great arteries {S,D,D}.

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia

2021 ◽  
Vol 25 (3) ◽  
pp. 218-228 ◽  
Author(s):  
Casey A. Quinlan ◽  
Gregory J. Latham ◽  
Denise Joffe ◽  
Faith J. Ross
Keyword(s):  
Blood Flow ◽  
Tetralogy Of Fallot ◽  
Pulmonary Blood Flow ◽  
Anesthetic Management ◽  
Ductus Arteriosus ◽  
Pulmonary Atresia ◽  
Systemic Circulation ◽  
Arterial System ◽  
Pulmonary Vasculature ◽  
Pulmonary Arterial

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

The real fate of pulmonary arteries after bidirectional superior cavopulmonary anastomosis: is there a need for concern?

1999 ◽  
Vol 9 (1) ◽  
pp. 6-10 ◽  
Author(s):  
Zdenek Slavik ◽  
Rodney C. G. Franklin ◽  
Rosemary Radley-Smith
Keyword(s):  
Blood Flow ◽  
Pulmonary Blood Flow ◽  
Pulmonary Arteries ◽  
Non Invasive ◽  
Morphological Heterogeneity ◽  
Cavopulmonary Anastomosis ◽  
In Series ◽  
Pulmonary Arterial ◽  
Arterial Development ◽  
Bidirectional Cavopulmonary Anastomosis

AbstractControversy remains about the growth of the pulmonary arteries following a bidirectional superior cavopulmonary anastomosis in children with complex cyanotic congenital cardiac malformations. This is partially due to the morphological heterogeneity of the patients, and partially due to methodological differences in series published so far. It is further complicated by the variable use, in different centres, of additional sources of pulmonary blood flow. We believe that the fate of these arteries preoperatively is significantly influenced by the amount of pulmonary blood flow and the initial size of the arteries. Separate assessment of the pulmonary arterial development postoperatively is recommended for those who, initially, had relatively small as opposed to larger than normal pulmonary arteries. Measurement of the diameters of both pulmonary arteries just prior to their first point of branching, together with the use of Z-score evaluation rather than the Nakata index, is discussed. It remains to be established whether, over time, the bidirectional cavopulmonary anastomosis is effective in developing adequately the pulmonary arteries in preparation for an ultimate total cavopulmonary connection, or even as isolated long-term palliation. A prospective, multi-institutional study involving sequential non-invasive assessment of pulmonary arterial development (using, for example, magnetic resonance imaging) is required to solve the outstanding problems.

Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

2021 ◽  
pp. 1-4
Author(s):  
Baher M. Hanna ◽  
Wesam E. El-Mozy ◽  
Sonia A. El-Saiedi
Keyword(s):  
Right Ventricular ◽  
Interventricular Septum ◽  
Pulmonary Atresia ◽  
Right Ventricular Outflow Tract ◽  
Rare Condition ◽  
Surgical Excision ◽  
Ventricular Outflow Tract ◽  
Outflow Tract ◽  
Ventricular Outflow Tract Obstruction ◽  
The Right

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

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