Pathology
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Fetopathology and developmental pathology of the embryo and fetus
Marta Ježová, Josef Feit et al.
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+ Atlas of fetal pathology
+ Congenital malformations
+ Physiological developement of an embryo and fetus, growth and changes of the external shape
+ Congenital malformations and congenital anomalies
+ Basic terminology
+ Etiology of congenital anomalies (CA)
+ Environmental causes of congenital anomalies, congenital infections
+ Rubella
+ Cytomegalovirus
+ Syphilis
+ Toxoplasmosis
+ Diabetes mellitus (DM) and pregnancy
+ Diabetic embryopathy
+ Diabetic fetopathy
+ Genetic causes of congenital malformations
+ Chromosomal abnormalities
+ Down syndrome
+ Klinefelter syndrome
+ Turner syndrome
+ Patau syndrome
+ Edwards syndrome
+ Triploidy
+ Single gene disorders
+ Tuberous sclerosis
+ Miscellaneous malformation syndromes, sequences and associations, unclassified
+ Sirenomelia (Mermaid syndrome)
+ Hydrops fetalis
+ Immune hydrops (erythroblastosis fetalis, hemolytic disease of the newborn)
+ Nonimmune hydrops
+ Hygroma colli cysticum
+ Pierre-Robin sequence
+ Caudal regression syndrome (sacral agenesis)
+ VACTERL association
+ Pathology of twinning
+ Complications of monochorionic diamnionic placentation
+ Chronic twin transfusion syndrome
+ Acute twin-twin transfusion
+ Complications of monochorionic monoamnionic placentation
+ Congenital malformations in twins
+ Chorangiopagus parasiticus (acardius)
+ Pathology of higher multiple gestations: triplets
+ Monsters
+ Monstra duplicia
+ Gemini monochoriati inequales
+ Duplicitas symmetros
+ Asymmetric dual monsters
+ Monstra simplicia
+ Congenital malformations of individual organ systems
+ Congenital malformations of the central nervous system
+ Congenital malformations of the spinal cord
+ Spina bifida
+ Myeloschisis (rachischisis posterior)
+ Congenital malformations of the brain
+ Anencephaly
+ Iniencephaly
+ Encephalocele
+ Arnold-Chiari malformation
+ Disorders of formation of the structures derived from the mediobasal prosencephalon
+ Migration disorders
+ Destructive lesions of fetal brain (disruptions)
+ Porencephaly
+ Hydranencephaly
+ Hydrocephalus
+ Dandy-Walker malformation
+ Intracranial non-neoplastic cysts
+ Arachnoid cyst
+ Neuroepithelial cyst
+ Congenital malformations of the respiratory system
+ Congenital cystic adenomatoid malformation (CCAM)
+ Lobar sequestration
+ Congenital lobar emphysema
+ Pulmonary hyperplasia
+ Pulmonary hypoplasia
+ Congenital pulmonary lymphangiectasia
+ Diaphragm and congenital malformations
+ Congenital malformations of kidneys and efferent urinary tracts
+ Renal agenesis
+ Variations of shape and position of the kidneys
+ Renal cystic disease
+ Infantile polycystic kidneys (ARKPD)
+ Renal dysplasia
+ Adult polycystic kidneys (ADPKD)
+ Autosomal dominant polycystic kidney disease in infant and fetus
+ Lower urinary tract obstruction
+ Posterior urethral valves
+ Congenital tubular renal dysgenesis
+ Urinary bladder
+ Hypospadia
+ Congenital malformations of genital system and somatosexual congenital disorders
+ Somatosexual disorders
+ Somatosexual disorders with normal karyotype
+ Pseudohermaphroditism masculinus
+ Androgen insensitivity syndrome, testicular feminization syndrome, female XY
+ Pseudohermaphroditism femininus
+ Congenital adrenal hyperplasia
+ Somatosexual disorders with pathologic karyotype
+ Congenital malformations of female genitalia
+ Congenital malformations of the internal genitalia
+ Congenital malformations associated with congenital malformations of urinary system
+ Congenital malformations of the vulva
+ Congenital malformations of the male genital organs
+ Cryptorchidism
+ Congenital malformations of penis and scrotum
+ Smith-Lemli-Opitz syndrome (SLOS)
+ Congenital tumors
+ Sacrococcygeal teratoma
+ Congenital cardiac rhabdomyoma
+ Congenital heart defects
+ Defects of heart septum
+ Atrial septal defects
+ Ventricular septal defects
+ AV septal defects
+ Anomalies of the great arteries
+ Transposition of the great arteries
+ Truncus arteriosus
+ Double-outlet right ventricle
+ Malformations of the valves
+ Tricuspid atresia
+ Stenosis and atresia of the mitral valve, the hypoplastic left heart syndrome
+ Stenosis of the pulmonary artery
+ Pulmonary atresia with intact ventricular septum
+ Tetralogy of Fallot
+ Aortic stenosis
+ Malformations of the aortic arch system
+ Persistence of ductus arteriosus
+ Coarctation of the aorta
+ Interruption of the aortic arch
+ Right-sided aortic arch
+ Malformations of the venous system
+ Anomalies of situs and heart position
+ Di George syndrome
+ Congenital malformations of the gastrointestinal tract
+ Oral cavity and the palate
+ Cleft lip and palate
+ Congenital defects of the esophagus
+ Atresia
+ Congenital esophagus stenoses
+ Congenital malformations of the stomach
+ Congenital malformations of the intestines
+ Atresia
+ Malrotation of the intestine
+ Remnants of the omphalomesentric duct
+ Anorectal malformations
+ Duplicatures
+ Defects of the abdominal wall
+ Omphalocele
+ Gastroschisis
+ Limb-body wall complex
+ Pentalogy of Cantrell
+ Developemental anomalies of the skeleton
+ Defects of growth of tubular bone and/or spine
+ Thanatophoric dysplasia
+ Diastrophic dysplasia
+ Abnormalities of density of cortical diaphyseal structure or metaphyseal modeling
+ Osteogenesis imperfecta
+ Limb malformations
+ Terminal transverse limb defects
+ Radial ray defects
+ Finger anomalies
+ Club foot
+ Constriction (amniotic) band syndrome
+ Placental inflammation
+ Ascending infections
+ Placental inflammation, acute
+ Hematogenous infections
+ Fetomaternal listeriosis
+ Pathology of the fetal membranes
+ Amniotic band syndrome
+ Meconium staining
+ Candida infection of the placenta
+ Pathology of the placenta
+ Normal placenta
+ Extrauterine gravidity
+ Gestational trophoblastic disease (GTD)
+ Hydatidiform mole
+ Complete mole
+ Partial mole
+ Invasive mola (mole proliferans, mole destruens)
+ Gestational choriocarcinoma
+ Placental site trophoblastic tumor
+ Placenta and major chromosomal abnormalities
+ Hydropic placenta
+ Pathology of the umbilical cord
+ Abnormal length
+ Short cord
+ Long cord
+ Insertion abnormalities
+ Abnormal coiling
+ Umbilical vessels pathology
+ Umbilical cord accidents which compromise the blood flow
+ Thrombosis of umbilical blood vessels
+ Umbilical cord inflammation
+ Acute funisitis
+ Subacute necrotizing funisitis
+ Miscellaneous rare cord lesions
Stenosis and atresia of the mitral valve, the hypoplastic left heart syndrome
Introduction

Mitral stenosis: the annulus is narrow, hypoplastic. The leaflets are thickend or fused with short chordae.

Mitral atresia:> the valve is completely absent with a dimple in the usual site of the valve, less common there is an imperforate membrane.

Stenosis or atresia of the mitral valve is the part of the hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a critical congenital heart defect with a small left ventricle and stenosis or atresia of the aortic and the mitral valve.

Macroscopic appearance
  • there is a wide spectrum of changes of the mitral valve, left ventricle and aorta
  • the combinations of the mitral stenosis and aortic atresia and combination of mitral and aortic stenosis are the most frequent
  • the whole aortic arch is strongly hypoplastic (the aorta is filiform)
  • the coronary arteries are supplied by reverse flow from the patent ductus arteriosus; the pulmonary artery is dilated
  • the left ventricle is always hypoplastic. With mitral atreis the left ventricle is extremely hypoplastic with thick walls and slitlike cavity or no cavity is present
  • with mitral stenosis a cavity in the small left ventricle is developed, the endocardium is fibrously thickened (fibroelastosis)
  • the right ventricle is hypertrophic pumping all the blood flow
Clinical signs
  • this critical defect manifests in the first days of life as the ductus closes by severe heart failure or shock
  • the affected infants uniformly die within few days without urgnet surgery
  • the defect is corrected in several steps (Norwood surgery)
  • after reconstruction the right ventricle works as the systemic ventricle but it cannot fully compensate the the normal left ventricle (right ventricle is a low-pressure pump)
  • in some cases this defect is inoperabile or could be corrected only by neonatal heart transplantation
  • individuals with hypoplastic left heart usually do not have any other congenital defects; hypoplastic left heart syndrome is not a typical part of other syndromes
Classification

Hypoplastic left heart syndrome is associated with premature (fetal) closure of foramen ovale in approximately 10% of the cases. The presence of intact atrial septum or highly restrictive (small) foramen ovale is a predictor of poor outcome in infants with hypoplastic left heart. Premature closure of foramen ovale is associated with coarctation of the aorta, an isolated form without any heart malformation is quite rare.

Pictures

Hypoplastic left heart syndrome, fibroelastosis of the left ventricle endocardium, fetus: Hypoplasia of the left ventricle, fibroelastosis, Macro, autopsy (72063)

Hypoplastic left heart with fetal closure of the foramen ovale: Hypoplastic left heart syndrome, Macro, autopsy (73189) Hypoplastic left heart syndrome, Macro, autopsy (73190) Hypoplastic left heart syndrome, Macro, autopsy (73191) Hypoplastic left heart syndrome, Macro, autopsy (73192) Hypoplastic left heart syndrome, Macro, autopsy (73193)

Another case of hypoplastic left heart with fetal closure of the foramen ovale: Hypoplastic left heart syndrome, Macro, autopsy (73194) Hypoplastic left heart syndrome, Macro, autopsy (73195) Hypoplastic left heart syndrome, Macro, autopsy (73196) Hypoplastic left heart syndrome, Macro, autopsy (73197) Hypoplastic left heart syndrome, Macro, autopsy (73198) Hypoplastic left heart syndrome, Macro, autopsy (73199)

Hypoplastic left heart: Hypoplastic left heart syndrome, Macro, autopsy (73292)

Hypoplastic left heart syndrome, ultrasound video: Hypoplastic left heart syndrome, Macro, autopsy (73664) Hypoplastic left heart syndrome, 1st trimester, Ultrasound, video (74011)

Hypoplastic left heart syndrome: Hypoplastic left heart syndrome, Macro, autopsy (73665) Hypoplastic left heart syndrome, Macro, autopsy (73666) Hypoplastic left heart syndrome, Macro, autopsy (73667) Hypoplastic left heart syndrome, Macro, autopsy (73668) Hypoplastic left heart syndrome, Macro, autopsy (73669) Hypoplastic left heart syndrome, Macro, autopsy (73670) Hypoplastic left heart syndrome, Macro, autopsy (73671) Hypoplastic left heart syndrome, Macro, autopsy (73672)

Case study
Hypoplastic left heart syndrome 1
Marta Ježová
History

24-week fetus, mitral atresia, aortic atresia, hypoplastic left heart syndrome.

Pictures

Hypoplastic left heart syndrome, base of the heart: Hypoplastic left ventricle, Macro, autopsy (72065)

Hypoplastic left heart syndrome, atresia of the mitral valve, left ventricle: Hypoplastic left ventricle, mitral atresia, Macro, autopsy (72062)

Hypoplastic left heart syndrome, hypertrophy of the right ventricle: Right ventricle in left heart hypoplasia, Macro, autopsy (72064)

Hypoplastic left heart syndrome, slit-like left ventricle at mitral atresia: Hypoplastic left heart, Macro, autopsy (72144)

Case study
Hypoplastic left heart syndrome 2
Marta Ježová
History

Immature 31-week newborn from twin pregnancy, mitral stenosis, aortic stenosis, hypoplastic left heart syndrome.

Pictures

Hypoplastic left heart syndrome: Hypoplastic left heart syndrome, Macro, autopsy (72371) Hypoplastic left heart syndrome, Macro, autopsy (72373)

Hypertrophy of the right ventricle: Hypoplastic left heart syndrome, Macro, autopsy (72375) Hypoplastic left heart syndrome, Macro, autopsy (72376)

Fibroelastosis of the left ventricle: Hypoplastic left heart syndrome, Macro, autopsy (72377)

Aortic valvular stenosis: Hypoplastic left heart syndrome, Macro, autopsy (72380)

Mitral stenosis: Hypoplastic left heart syndrome, Macro, autopsy (72372)