Tetralogy of Fallot (TOF) is a congenital heart defect characterized by four abnormalities: ventricular septal defect, pulmonary stenosis, overriding aorta, and right ventricular hypertrophy. It has been successfully repaired surgically since the 1950s. Current surgical repair in infancy has excellent outcomes, aiming to relieve right ventricular outflow tract obstruction. Long term complications can include pulmonary regurgitation and right heart dysfunction, but most TOF patients now survive well into adulthood thanks to advances in diagnosis and treatment.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle.
TGA is a complex congenital heart disease.Understanding the anatomy,physiology,surgery and anaesthetic management is very important for patient's better outcome.This ppt explains all these points in detail.
A 30-minute talk, presented as part of the weekly teaching activities in Alder Hey Children's Hospital (Liverpool, UK). It addresses PDA evaluation in children - starting with embryology & anatomy with the basis behind physiological closure versus patency after birth. What is the role of echo study in diagnosing/evaluating PDA? Modes used with some clear movies? Its limitations?
preop TEE assessment of atrial septal defect is very important for making decision for device closure, properly assessed adequate rims of ASD will reduce risk of device embolization to almost nil.
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle.
TGA is a complex congenital heart disease.Understanding the anatomy,physiology,surgery and anaesthetic management is very important for patient's better outcome.This ppt explains all these points in detail.
A 30-minute talk, presented as part of the weekly teaching activities in Alder Hey Children's Hospital (Liverpool, UK). It addresses PDA evaluation in children - starting with embryology & anatomy with the basis behind physiological closure versus patency after birth. What is the role of echo study in diagnosing/evaluating PDA? Modes used with some clear movies? Its limitations?
preop TEE assessment of atrial septal defect is very important for making decision for device closure, properly assessed adequate rims of ASD will reduce risk of device embolization to almost nil.
The lecture is for medical student. It is from Dr RUSINGIZA Emmanuel, MD, senior lecture at UR( UNIVERSITY OF RWANDA) .
It will help to understand heart diseases in newborn, infants and children.
most common congenital cyanotic heart disease.one of the conotruncal family of heart lesions.. It accounts for 7 to 10% of all congenital heart abnormalities.
Tetralogy of Fallot (TOF) is a congenital heart defect, which has four anatomical components:
Anterior malalignment ventricular septal defect (VSD)
Aortic override over the muscular septum
Variable degrees of subvalvar, valvar, and supravalvar pulmonary stenosis
Right ventricular (RV) infundibular narrowing and RV hypertrophy
Most common cyanotic heart defect seen in children beyond infancy, accounting for a third of all congenital heart disease (CHD) in this age group
Tetralogy of Fallot (TOF) is a congenital heart defect, which has four anatomical components:
Anterior malalignment ventricular septal defect (VSD)
Aortic override over the muscular septum
Variable degrees of subvalvar, valvar, and supravalvar pulmonary stenosis
Right ventricular (RV) infundibular narrowing and RV hypertrophy
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New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Anti ulcer drugs and their Advance pharmacology ||
Anti-ulcer drugs are medications used to prevent and treat ulcers in the stomach and upper part of the small intestine (duodenal ulcers). These ulcers are often caused by an imbalance between stomach acid and the mucosal lining, which protects the stomach lining.
||Scope: Overview of various classes of anti-ulcer drugs, their mechanisms of action, indications, side effects, and clinical considerations.
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
2. History
Tetralogy of Fallot was first described by Niels Stenson in
1671, although its precise anatomical description was
elegantly illustrated by William Hunter at St Georges
Hospital Medical School in London in 1784.
His description of a large outlet ventricular septal defect
together with subpulmonary and pulmonary valve stenosis,
and its resulting physiology, was refined by Etienne-
Louis Fallot in 1888 in his description of L’anatomie
pathologique de la maladie bleu, but the term
tetralogy of Fallot is attributed to Canadian Maude
Abbott in 1924.
3.
4. Epidemiology
About 3·5% of all infants born with a congenital heart
disease have tetralogy of Fallot, corresponding to one in
3600 or 0·28 every 1000 livebirths, with males and females
being affected equally.
Its precise cause is unknown, as for most congenital heart
diseases.
Most cases seem sporadic, although the risk of recurrence
in siblings is about 3% if there are no other affected first-
degree relatives.
5.
6. Genetic factors
Monogenic or polygenic mode of inheritance.
Autosomal dominant mode of inheritance with reduced
penetrance,
An autosomal recessive mode of inheritance,
Multifactorial cause supporting the hypothesis that the
genetic cause of TOF is heterogeneous.
Environmental factors
Maternal diabetes [threefold increased risk], Retinoic acids,
Maternal phenylketonuria (PKU), and Trimethadione
7. Syndromes and associations
DiGeorge/Velocardiofacial syndrome, Down syndrome,
Alagille syndrome, Cat's-eye syndrome, recombinant
chromosome (or San Luis Valley) and Kabuki syndromes,
CHARGE and VATER/VACTERL associations, Poland
syndrome and Goldenhar’s syndrome(oculo-auriculo-
vertebral dysplasia).
Microdeletion on 22q11….seen in
15–35% of TOF patients;
45% of TOF + PA;
65% of those with TOF + APV.
8. Embryology
The aorta and pulmonary artery start as a single tube the
truncus arteriosus which is then divided by the spiral
septum.
The spiral septum grows down to and attaches to the
ventricular septum which isolates the left and right
ventricles as well as isolating the aorta and pulmonary
artery.
However, if the spiral septum is not in the midline,
but rather shifted over towards the right side of the
heart, then what would happen?
The opening to the aorta would be large The opening to the
pulmonary would be small The spiral septum would “miss”
the ventricular septum.
9.
10. One abnormality results in 3 of the “defects” in TOF:
"overriding” aorta (it opens up to both the right and left
ventricles) pulmonary stenosis (the pulmonary artery is
small and narrowed) VSD (because the spiral and
ventricular septa do not line up and connect).
The RV has to work extra hard to
pump blood into the pulmonary artery and therefore
hypertrophies to accommodate the extra work resulting
in RVH.
The pulmonary stenosis (PS) determines the degree of
severity of this defect. Pulmonary perfusion depends on
the size of the PS.
11. Anatomy
Anterior and cephalad deviation of the infundibular
septum.
This results in a malaligned ventricular septal defect (VSD),
with the aortic root overriding the defect and leading to
subsequent right ventricular outflow obstruction.
VSD is most commonly a single large malaligned subaortic
defect located in the perimembranous region of the septum.
The VSD can extend into the muscular septum.
There are rarely other muscular ventricular septal defects.
12. RVOT Obstruction
The right ventricular outflow obstruction is often at multiple
levels.
The anterior and cephalad deviation of the infundibular septum results in
subvalvar obstruction
Hypertrophy of muscular bands in this region can further accentuate
subvalvar obstruction
The pulmonary valve annulus is usually hypoplastic, although in some
instances it is of normal size
The pulmonary valve itself is frequently bicuspid and stenotic
It is not uncommon to identify an area of supravalvar
narrowing in the main pulmonary artery at the sinotubular
ridge.
There may also be further obstruction at the branch
pulmonary arteries.
These may be diffusely hypoplastic or have focal areas of
stenosis, most commonly at the proximal branch pulmonary
arteries.
13.
14.
15.
16. Overriding aorta
Aorta is displaced to the right over the VSD rather than the left
ventricle.
This results in blood flow from both ventricles into the aorta.
The degree of aortic override of the VSD can vary widely and is
one of the major factors used by some groups to differentiate
between TOF and DORV.
If one defines double outlet right ventricle as the presence
of aortic/mitral valve fibrous continuity, then the degree of
override is not relevant to diagnosis.
If, however, one defines double outlet right ventricle as a
condition with greater than 50 percent aortic override, then,
by definition, the degree of aortic override in TOF is limited.
21. Pathophysiology
The VSD is almost always large and non-restrictive in TOF,
ensuring that the pressure is equal in the two ventricles.
Consequently, the loud systolic murmur typical in affected
infants originates from the dynamic narrowing of RVOT.
The direction and magnitude of flow through the defect
depends on the severity of the obstruction of RVOT.
Most patients have adequate pulmonary blood flow at birth
but develop increasing cyanosis during the first few weeks
and months of life.
22. One of the physiologic characteristics of TOF is that the RVOT
obstruction can fluctuate.
An individual with minimal cyanosis can develop a dynamic
increase in right ventricular outflow tract obstruction with a
subsequent increase in right-to-left shunt and the development
of cyanosis.
In the most dramatic situation, there can be near occlusion of the
right ventricular outflow tract with profound cyanosis.
These episodes are often referred to as "tet spells" or
"hypercyanotic spells".
23. Typical spell begins with progressive increase in rate and
depth of respiration and culminates in paroxysmal
hyperapnea, deepening cyanosis, limpiness, syncope and
occasionally in convulsions, CVA and death.
Peak incidence in between second and sixth month of life.
Spells typically initiated by stress of feeding, crying or a
bowel movement, particularly after awakening from deep
sleep.
Mechanisms -
Acceleration in heart rate
Increase in C.O. and venous return
Increase in R→L shunt
Vulnerable respiratory centre
Infundibular contraction
27. Clinical features
Wide due to variable severity of RV outflow obstruction
USGfetal diagnosis
Newborn & infants…cyanosis and systolic murmur.
Acyanotic TOFmild pulmonary overcirculation rarely
CHF unless there is a large PDA or aortopulmonary
collateral arteries
Pregnancy…poorly tolerated … gestational decrease in
SVR increases R-L shunt & the labile SVR during labor,
delivery leads to abrupt hypoxemia…..high fetal wastage /
dysmature offspring.
28. Examination
Cyanosis, Clubbing
JVP is normal
Arterial pulses …normal in uncomplicated TOF
Wide pulse pressure (arterial diastolic runoff)….
aortopulmonary collaterals, palliative surgical shunt or
PDA
Accentuated precordial RV impulse
LV impulse will not be hyperactive (normal cardiac output)
S2 single& loud (anterior, dextroposed aorta)
S3/S4 are unusual
Aortic ejection click
29. Systolic murmur…crescendo-decrescendo @ LUSB.
The intensity of the murmur inversely parallels the degree
of pulmonic obstruction.
Diastolic murmurs are unusual..Rarely AR murmur
TOF with PA…..no harsh, obstructive precordial murmurs
A harsh diastolic murmur, with a harsh murmur of PS,
[harsh sawing, to-and-fro murmur ] ……TOF and APV
syndrome
Continuous murmurs….PDA, aortopulmonary collaterals,
may be best heard in the back.
33. Echocardiography
2-D echocardiography provides the cornerstone for
noninvasive diagnosis of TOF.
Doppler analysis provides further data regarding
hemodynamic characteristics.
Study should be completed in a segmental and consistent
manner that allows complete definition of the desired
information.
The coronary arteries are also readily apparent in PSAX view
slightly superiorly.
The origin and course of the left and right coronary arteries
should be determined, paying special attention to whether any
vessels take an anterior course across the infundibulum.
Correct definition of the coronary artery anatomy was possible
in most patients with a sensitivity of 82%, specificity of 99%,
and accuracy of 98.5% in a study by Need et al.
34.
35.
36. LAD
RCC
NCC
RCA
LCx
Pulm.
Valve
MPA
The anomalous LAD crossing the RVOT in TOF is identified when the transducer
is swept superiorly in the parasternal short -axis view .
This allows visualization of the anomalous LAD that is situated anterior to the
RVOT
37. Cardiac Catheterization
With the evolution of noninvasive technology, the
indications for diagnostic cardiac catheterization have
diminished substantially.
Nonetheless, invasive study to obtain both hemodynamic
and anatomic information is, on occasion, helpful prior to
deciding on surgical strategy.
Foremost goal …. clarification or better definition of
anatomic characteristics, such as pulmonary arterial or
coronary arterial anatomy.
Coronary artery anatomy … either by aortic root angio,
selective coronary artery injection, or a combination of
both.
38.
39.
40. A: An ascending aortogram in the LAO projection demonstrates a right anterior
descending coronary artery arising from the right coronary artery.
B: A selective left coronary injection shows filling of only the circumflex coronary artery.
46. The goal of management of TOF patients is to allow total surgical
correction with minimal mortality and morbidity and to prevent
or treat complications.
Most newborns with TOF do not have ductal-dependent
pulmonary blood flow and may be followed without specific early
intervention.
Depending of the severity of obstruction within the RV outflow
tract, an infusion of prostaglandin may be initiated to preserve
ductal patency, and provide a stable source of flow of blood to the
lungs.
IE Prophylaxis as per recommendations.
47. Hypercyanotic spells
Treatment aims @ lowering impedance to pulmonary flow and
further increasing systemic vascular resistance.
Rx…..Oxygen, Volume expansion, Sedation with morphine or
ketamine, and, if needed, vasopressors[phenylephrine]
Refractory to above …..
Transfusion of whole blood/red cells
Balloon angioplasty of pulmonary annulus
Emergent surgical palliation or repair
Propranolol …having some efficacy in minimizing or
extinguishing the occurrence of spells. It may be used for patients
who are awaiting surgical intervention or who have a medical
contraindication to either complete repair or aortopulmonary
shunt.
48. Timing of Surgery
Palliative shunts were initially reserved for infants, and
intracardiac repair for children, but the excellent results of
intracardiac repair for neonates and infants have made it
the current treatment of choice for all patients with TOF in
most centers.
Surgery is usually performed electively in the first year of
life and can be performed in the first three months, if
necessary.
Intracardiac repair is also performed in asymptomatic
acyanotic infants (pink variant) with a low morbidity and
mortality; this approach may allow normal growth of the
RVOT and pulmonary annulus.
49. Contraindications for repair in early infancy
Palliation initially
LAD from RCA crossing infundibulum
Severely hypoplastic PAs
Pulmonary atresia
50. Aim of surgery
Relieving all possible sources of RVOTO.
If possible, pulmonary valve function is preserved by avoiding
a transannular patch.
Closure of VSD (dacron patch)
To relieve RVOTOpulmonary valvotomy, the insertion of an
outflow tract patch or a transannular patch are often required.
Surgery during early infancy, when the pulmonary annulus is
markedly stenotic, frequently requires the insertion of a long
and wide transannular patch.
Consequently, most patients acquire PR as a result of the
repair. PR may be well tolerated by many in the early
postoperative years, but in the long term chronic PR is
associated with reduced exercise capacity, RV dilatation,
ventricular arrhythmias, and sudden death.
51. PA anatomy assessment
1) McGoon ratio: (Diameter of RPA/DAo + Diameter of LPA/DAo)
Normal 2.1
Adequate for VSD closure 1.2
Inadequate <0.8 for VSD closure
2) Nakata Index:(CSA of RPA + CSA of LPA)/BSA
Normal value > 200 mm2/m2
> 150 mm2/m2 is adequate.
(Not usable preoperatively when MAPCAs are the major source of PBF & one-
stage unifocalization + full repair is planned).
3) Total Neo-Pulmonary Artery Index (TNPAI) = APC index + Nakata Index
APC index is the sum of CSA of all usable APCs/BSA
>250 - suitable for one-stage repair including VSD closure (These pts. have low
RV/LV pressure ratio postoperatively).
4) Z- score – no’s of SD PA size is away from the mean for children of same age and
sex.
Neaton’s number, Natio index, Blackstone index and Kugelberg angiographic
classification.
52.
53. Post Repair for TOF
Current surgical survival, even for symptomatic infants <3
months of age, is excellent.
Hospital and 1-month survival rates of 100% have been
reported.
Earlier age at repair (<1 year of age) did not adversely affect
the rate of reintervention; so primary repair should be
regarded as the preferred management strategy.
Twenty-year survival for hospital survivors, irrespective of
management strategy, was 98% for patients who have TOF
with PS and slightly lower for patients with PA, reflecting
the overall excellent long-term survival of these patients.
55. Conclusions
Tetralogy of Fallot is the most common form of cyanotic
congenital heart disease, and one of the first to be
successfully repaired by congenital heart surgeons.
Since the first procedures in the 1950s, advances in the
diagnosis, perioperative and surgical treatment, and
postoperative care have been such that almost all those
born with tetralogy of Fallot can now expect to survive to
adulthood.
The care of children with tetralogy of Fallot and their
transition to adult life has been a success of modern
medicine.
Most of them now survive early repair and have an
essentially normal childhood.
Editor's Notes
GLH – Green Lane hospital, Auckland (NZ); UAB- University hospital Alabama(US)