William Harvey (1578-1657) : De motu cordis et sangunis 1628 William Harvey, one of the most gifted pupils of Fabricius (1578-1657), combined all these individual findings with the results of his own research to form the general picture of what we today call the circulation of the blood. But even he had no clear idea of the circulation in the region of the capillaries. This section was explained and described for the first time by Malpighi in 1661, after he had viewed a frog's lung under a microscope. In any event, it is the year 1628, in which Harvey published his classic work De motu cordis et sangunis, that we can call the birth-year of cardiology.
1896 closure of a stab would in the right ventricle in Frankfurt am Main by Prof. Dr. Ludwig Rehn. Patient developed empyema but eventually survived.
(Johann von Mikulicz,in) 1903 assigned Ferdinand Sauerbruch (1875-1951) to work on open chest surgery, negative pressure chamber Sauerbruch on October 1, 1903 came to Johannes von Mikulicz-Radecki (1850-1905) in the university surgical clinic at Breslau. As an ambulant physician (Volont ä rarzt) Sauerbruch here conducted his first attempts at thoracic surgery and started to work on his most important invention: a negative-pressure chamber enabling the safe opening of the chest while conducting a pneumothorax. After series of test on animals, Sauerbruch proudly presented his contraption to Mikulicz-Radecki – but the experiment misfired. Mikulicz-Radecki felt insulted and dismissed Sauerbruch from his clinic. Sauerbruch continued his experiments at a private clinic and eventually won the acceptance of Mikulicz-Radecki. Together they presented the under-pressure apparatus at the surgical congress in Berlin. The first operation conducted on a human failed, however, but subsequently thoracical surgery using the under-pressure chamber advanced rapidly. On June 8, 1905 Sauerbruch was habilitated with the thesis Experimentelles zur Chirurgie des Brustteils der Speisr ö hre. Only six days later he attended the funeral of his teacher Mikulicz-Radecki.
Helen Brooke Taussig , M.D. , ( 1898 - 1986 ) was an American cardiologist , working in Baltimore and Boston, who founded the field of pediatric cardiology. Notably, she helped develop the Blalock-Taussig shunt in cooperation with Dr. Alfred Blalock and Vivien Thomas , to treat blue baby syndrome Blalock-Taussig shunt , first performed by Taussig and Dr. Alfred Blalock on an 11-month old baby girl on November 29,1944. Taussig wrote the book Congenital Malformations of the Heart in 1947 In 1959, she was one of the first women to be awarded a full professorship at Johns Hopkins University
First successful surgical repair of the heart on September 2 , 1952 by Walton Lillehei ( 1918 – 1999 ) under hypothermia Lillehei performed surgeries using cross-circulation, in which a donor was hooked up nearby to take up the pumping and oxygenation functions of the patient as he was being operated on. Using this technique, Lillehei led the team that performed successful repair of a ventricular septal defect on March 26 , 1954 . Although the repair was successful, the patient, 13-month old Gregory Glidden, died 11 days later of suspected pneumonia. Lillehei and his team continued to use cross-circulation for a total of 44 open-heart operations in the following year, of which 32 patients survived. These surgeries included the first repairs of the and tetralogy of Fallot . Working with Dr. Richard A. DeWall, Dr. Lillehei developed the first clinically successful bubble oxygenator which supplanted the use of cross-circulation in 1955 . The availability of the simple Lillehei-DeWall oxygenator allowed for tremendous growth of open heart surgical programs the world over. (With Gibbon ’ s HLM on May 6, 1953, surgery using the heart-lung machine was successfully performed on the first human, 18-year-old Cecilia Bavolek, to close a hole between her upper heart chambers. Gibbon ’ s bypass machine was first employed at the Mayo Clinic on a five year old girl on March 23, 1955. Dr. Kirklin was the cardiac surgeon.) In 1958 , Lillehei was responsible for the world's first use of a small, external, portable, battery-powered pacemaker , invented at his behest by Earl Bakken (whose then-small company, Medtronic , designed and repaired electronics for the University of Minnesota hospital). Lillehei also developed and implanted the world's first prosthetic heart valves : the Lillehei-Nakib toroidal disc ( 1966 ), the Lillehei-Kaster pivoting disc (1967), and the Kalke-Lillehei rigid bileaflet prosthesis ( 1968 ).
Jacqueline Noonan (1921-) Genetics of Noonan syndrome Noonan syndrome was recognized early on as an autosomal dominantly inherited disorder, but the majority of cases appeared to be sporadic. Dr. Allanson in 1985 (24) made the important observation that the phenotype of Noonan syndrome changes significantly over time. She found if photographs of parents taken at the same age as their affected child and compared they would frequently suggest one of the parents also had Noonan syndrome. As is common in autosomal dominantly inherited disorders, there is often great variability in expression and mild cases may go unrecognized. In 1994 Jamison et. al (25) studied some familial cases of Noonan syndrome and were able to map the gene for Noonan syndrome to the long arm of chromosome 12. Not all families with Noonan syndrome showed linkage to this chromosome suggesting that there was more than one genetic cause for Noonan syndrome. Recently, using new information provided by the human genome project, the group headed by Dr. Bruce Gelb (26), identified the Noonan syndrome gene on chromosome 12. This gene is called PTPN-11 and regulates the product of a protein named SHP-2. This is a protein essential in several intracellular single transduction pathways that control a number of developmental processes including cardiac semilunar valvular genesis. Valvular pulmonary stenosis with a dysplastic pulmonary valve is the most common lesion found in Noonan syndrome. This suggested that the PTPN-11 gene would be a likely candidate since mice with a mutated gene often had aortic and pulmonary stenosis. Patient studies included two moderate size families who had shown linkage to chromosome 12. All affected members showed missence mutations in the PTPN-11 gene. An additional 22 unrelated individuals with Noonan syndrome representing sporadic or small families were also studied. Half of these had missence mutations in PTPN-11 similar to the family studies. A more recent report (27) now includes studies in 119 individuals with Noonan syndrome. 54 of the 119 or 45% were demonstrated to have mutations. There was a higher prevalence of mutations in familial cases than in sporadic. Among those patients with Noonan syndrome and pulmonary valve stenosis, PTPN-11 mutations were found in 70.6% while those with Noonan syndrome and hypertrophic cardiomyopathy showed a lower incidence of 5.9%. In the not too distant future, it should be possible to screen for PTPN-11 in other Noonan syndrome like conditions such as Cardio-facio-cutaneous, Leopard and Noonan-neurofibromatosis syndromes. It is also likely that in the future linkage studies in other families with Noonan syndrome will map to a specific chromosome and other genes will be identified that are responsible for those cases of Noonan syndrome not due to a mutation of the PTPN-11 gene. For the first time, there is hope that a genetic test will be available to make a firm diagnosis of Noonan syndrome. Up to the present time the diagnosis relies on clinical findings alone.
Scientific medicine begins one of the most gifted pupils of Fabricius (1578-1657), to form the general picture of what we today call the circulation of the blood. But even he had no clear idea of the circulation in the region of the capillaries. William Harvey
Prof. Dr. Ludwig Rehn 1896 closure of a stab would in the right ventricle in Frankfurt am Main by Prof. Dr. Ludwig Rehn. Patient developed empyema but eventually survived.
Ferdinand Sauerbruch (1875-1951) A negative-pressure chamber enabling the safe opening of the chest while conducting a pneumothorax was deviced After series of test on animals, on October 1, 1903
( 1898 - 1986 ) was an American cardiologist , working in Baltimore and Boston, who founded the field of pediatric cardiology. Notably, she helped develop the Blalock-Taussig shunt in cooperation with Dr. Alfred Blalock and Vivien Thomas , to treat blue baby syndrome Helen Brooke Taussig, M.D.
the book- Congenital Malformations of the Heart in 1947 Blalock-Taussig shunt , first performed on an 11-month old baby girl on November 29,1944.
In 1959, she was one of the first women to be awarded a full professorship at Johns Hopkins University
First successful surgical repair of the heart on September 2 , 1952 by under hypothermia performed surgeries using cross-circulation, in which to take up the pumping and oxygenation functions of the patient as he was being operated on March 26 , 1954 Walton Lillehei ( 1918 – 1999 )
Jacqueline Noonan (1921-) Genetics of Noonan syndrome
The ductus arteriosus closed by its contraction of muscular wall, which resulted by bradykinin
By increasing in pulmonary blood flow, pulmonary venous return increased, combined with a decrease in pressure within the right atrium, resulted in a closing of foramen ovale
Close of the circulation in infant after birth
—— Functional close: after birth
—— Anatomically close: 5 ~ 7 months
—— Functional close: within 24h after birth (term infant 80%)
—— Anatomically close: 3 month (80%)
one year (95%)
—— atresia to forming ligaments
Placenta : source of oxygen for the fetus
To compare circulation between prior and after birth 返回 A : fetal period B : after birth Gas exchange by matrix systemic circulation Gas exchange by pulmonary circulation Blood oxygen content : Mixing or upper: heart /brain ﹥ lower body Separation of the venous and arterial blood PFO/PDA/PDV Close The same pressure of the aterail and pulmonary High pulmonary resistance The lower of the pulmonary pressure and resistance The burden of the right ventricle higher The burden of the left ventricle higher A B
Knee-chest Position Child with a cyanotic heart defect squats (assumes a knee-chest position) to relieve cyanotic spells. Some times called “tet” spells. Ball & Bindler Nurse puts infant in knee-chest position. Whaley & Wong
Specific examination: imaging/ hemodynamics/intervention