Tetralogy of fallot with absent left pulmonary artery: Report of a case with anomalous development of the right hilar vasculature and nonfunctioning right lung

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.

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Hidden pulmonary arteries in tetralogy of Fallot and pulmonary artery pressure in patients operated with a pulmonary artery

BMC Cardiovascular Disorders ◽

10.1186/s12872-021-01877-y ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Mohammadreza Edraki ◽

Bahram Ghasemzadeh ◽

Kambiz Keshavarz ◽

Ahmadali Amirghofran ◽

Hamid Mohammadi ◽

...

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Vein ◽

Ductus Arteriosus ◽

Pulmonary Arteries ◽

Left Pulmonary Artery ◽

Pulmonary Artery Hypertension ◽

Surgical Correction ◽

Right Pulmonary Artery ◽

The Right

Abstract Introduction The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery. Material and methods This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful. We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery. Results The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6–9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction. However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients. Conclusion The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

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Existence of Hidden Pulmonary Arteries in Tetralogy of Fallot and pulmonary artery hypertension in patients Operated with one Pulmonary Artery

10.21203/rs.3.rs-58127/v2 ◽

2020 ◽

Author(s):

Mohammadreza Edraki ◽

Bahram Ghasemzadeh ◽

Kambiz Keshavarz ◽

Ahmadali Amirghofran ◽

Hamid Mohammadi ◽

...

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Vein ◽

Ductus Arteriosus ◽

Pulmonary Arteries ◽

Left Pulmonary Artery ◽

Pulmonary Artery Hypertension ◽

Surgical Correction ◽

Right Pulmonary Artery ◽

The Right

Abstract Introduction: The absence of a pulmonary artery is a rare congenital anomaly that occurs isolated or with other congenital cardiac disorders, particularly tetralogy of Fallot (TOF); meanwhile, a hidden pulmonary artery might exist and originate from a closed ductus arteriosus (DA), which can be stented to reach the artery.Material and methods: This prospective study describes cardiac catheterization of nine TOF patients diagnosed with the absence of the left pulmonary artery before the operation. The patients were stratified into three groups: group one, whose closed DA was found and connected to the hidden pulmonary artery with a stent; group two, whose hidden pulmonary arteries were found via the pulmonary vein angiography; and group three, for whom we could not find the remnant of the DA, or our attempt to stent the DA to the hidden pulmonary artery was not successful.We also evaluated outcomes of six other surgically-corrected TOF patients who were operated with the absent left pulmonary artery.Results: The first group included the patients aged 1, 24, and 30 months, whose CT angiography 6-9 months after stenting showed acceptable left pulmonary artery diameter for surgical correction, and the pulmonary vein angiography of the second group showed a hidden left pulmonary artery with a suitable diameter for surgical correction.However, we were unable to find or stent the DA of group three patients, aged 12, 38, 60, and 63 months. Earlier Angiography might have increased the chance of access to the hidden vessel. Apart from these three groups, follow-ups of six other patients previously corrected with only the right pulmonary artery revealed pulmonary artery hypertension in all patients.Conclusion: The concealed pulmonary artery might be found, and stenting of the closed DA to it might be performed to improve the diameter of the diminutive pulmonary artery. This procedure may allow TOF total surgical correction with two pulmonary arteries. Besides, pulmonary vein angiography can reveal the hidden pulmonary artery.

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“Absent” left pulmonary artery with tetralogy of Fallot

Journal of Thoracic and Cardiovascular Surgery ◽

10.1016/s0022-5223(19)42957-7 ◽

1968 ◽

Vol 55 (3) ◽

pp. 333-336 ◽

Cited By ~ 4

Author(s):

Eugene Wallsh ◽

Edmund H. Reppert ◽

Eugenie F. Doyle ◽

Frank C. Spencer

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Left Pulmonary Artery ◽

Absent Left Pulmonary Artery

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Absent left pulmonary artery and double aortic arch in tetralogy of Fallot: reconstruction using homograft saphenous vein or iliac artery

Interactive Cardiovascular and Thoracic Surgery ◽

10.1510/icvts.2008.188201 ◽

2008 ◽

Vol 8 (2) ◽

pp. 277-279 ◽

Cited By ~ 3

Author(s):

S. Talwar ◽

A. Gupta ◽

S. K. Choudhary ◽

B. Airan

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Tetralogy Of Fallot ◽

Iliac Artery ◽

Saphenous Vein ◽

Left Pulmonary Artery ◽

Double Aortic Arch ◽

Absent Left Pulmonary Artery

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Tetralogy of Fallot with Absent Pulmonary Valve Syndrome with Absent Left Pulmonary Artery - A Rare Presentation

Bangladesh Heart Journal ◽

10.3329/bhj.v35i1.49148 ◽

2020 ◽

Vol 35 (1) ◽

pp. 74-77

Author(s):

Abul Kalam Shamsuddin ◽

Prodip Kumar Biswas ◽

Muhammad Ishtiaque Sayeed Al Manzoo ◽

Md Abul Kalam Azad ◽

Md Nurul Akhtar Hasan ◽

...

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Pulmonary Valve ◽

Postoperative Mortality ◽

Left Pulmonary Artery ◽

Absent Pulmonary Valve ◽

Congenital Cardiac Anomaly ◽

Ct Angiogram ◽

Absent Left Pulmonary Artery ◽

Absent Pulmonary Valve Syndrome

Absent left pulmonary artery with Tetralogy of Fallot (TOF) with absent pulmonary valve syndrome (APVS), is a rare congenital cardiac anomaly. Here we present such a case of A 2 year 11 month old girl with cyanosis, exertional dyspnoea. Her diagnosis is confirmed by echocardiography and CT angiogram. There are very few cases have been reported till date with high postoperative mortality. Although per operative decision making was challenging regarding pulmonary valve and size of the RPA, we performed ICR with RPA reductionplasty and creation of monocuspid pulmonary valve with success. As it is a rare association and we have overcome the hindrance we came across per operatively, we are reporting this case. Bangladesh Heart Journal 2020; 35(1) : 74-77

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Outcome at 26 Years After Repair of Fallot’s Tetralogy With Absent Left Pulmonary Artery

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135120932509 ◽

2020 ◽

Vol 11 (5) ◽

pp. 661-663

Author(s):

Guglielmo Stefanelli ◽

Marco Meli ◽

Rosaria Nardelli ◽

Emilio Chiurlia ◽

Luca Weltert

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Left Pulmonary Artery ◽

Peripheral Oxygen Saturation ◽

Term Outcome ◽

Long Term Outcome ◽

Lung Repair ◽

Lung Scan ◽

The Right

We report long-term outcome after two-stage, “one lung repair” in a four-year-old boy with tetralogy of Fallot and congenital absence of the left pulmonary artery. The operation was carried out two years after a palliative aortopulmonary central shunt and was uneventful. Twenty-six years later, the patient is in excellent clinical condition, with normal peripheral oxygen saturation. A recent radionuclide lung scan and cardiac magnetic resonance imaging show the pulmonary flow entirely directed into the right lung. In selected cases, the long-term prognosis of patients with tetralogy of Fallot and true absence of left pulmonary artery after “one lung repair” may be excellent.

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Intrapulmonary artery septation for unilateral absence of the pulmonary artery

Asian Cardiovascular and Thoracic Annals ◽

10.1177/02184923211013998 ◽

2021 ◽

pp. 021849232110139

Author(s):

Junichi Koizumi ◽

Tomoyuki Iwase ◽

Shigeto Tsuji ◽

Takuya Goto ◽

Kotaro Oyama ◽

...

Keyword(s):

Pulmonary Artery ◽

Tetralogy Of Fallot ◽

Left Pulmonary Artery ◽

Post Surgery ◽

Pulmonary Artery Reconstruction ◽

Artery Reconstruction ◽

Absent Pulmonary Artery ◽

Absent Left Pulmonary Artery ◽

Tetralogy Of Fallot Repair ◽

Blalock Taussig Shunt

We describe a seven-month-old boy with tetralogy of Fallot and an absent left pulmonary artery. Due to the diminutive size of the left pulmonary artery, we performed a native tissue left pulmonary artery reconstruction and intrapulmonary artery septation procedure with a left modified Blalock-Taussig shunt. After confirming left pulmonary artery growth, the patient underwent tetralogy of Fallot repair, removal of septation patch, and division of the Blalock-Taussig shunt. Nine months post-surgery, we confirmed his balanced lung perfusion (R/L ratio 6:4). The intrapulmonary artery septation procedure would be suitable for both the resuscitation and reconstruction of the hypoplastic absent pulmonary artery.

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